After reading such a long statement, running out of breath, going over it and getting overwhelmed, it is time to take a look at it and grasp at something very characteristic. A woman with pancytopenia. A bone marrow showing massive infiltration by immature elements that are proliferating non-stop...with Auer's canes? Acute promyelocytic leukemia. Those rods are the fusion of primary granules of developing myelocytes. Robin&Cotran say so on page 696 of their book "General Pathology" [1]. It is also said by Carr and Rodak in their book "Atlas of clinical hematology" on page 157 [2].

A 43-year-old woman consults her primary care physician for fatigue, gingivorrhea and petechiae. Laboratory tests showed anemia of 8 g/dL, thrombopenia of 4000/microliter and leukopenia of 1200/microliter with absolute neutropenia. The coagulation study showed APTT elongation (43″), decreased prothrombin activity (55%), hypofibrinogenemia (98 mg/dl) and presence of high concentration of D-Dimer and fibrin monomers. She is referred for urgent hematologic workup and a bone marrow aspirate is performed showing massive infiltration by immature elements with cleaved nuclei and Auer rods in the cytoplasm. What is the most likely diagnosis in this patient?

Postremission therapy in acute promyelocytic leukemia: room for improvement?

A 29-year-old white man presented to the hospital with a 3-week history of fatigue, generalized weakness, gingival swelling and bleeding, and headaches. Laboratory work revealed WBC 30.4 k/μL, hemoglobin 7.9 g/dL, and platelets 16 k/μL. The WBC differential showed 64% blasts and 24% promyelocytes. Coagulation studies revealed prothrombin time (PT) 13.5 seconds, internationalized normalized ratio (INR) 1.3, fibrinogen 199 mg/dL, D-dimer greater than 1.0 μg/mL, and fibrin split products greater than 40 μg/mL. A bone-marrow aspirate with biopsy was performed, yielding the diagnosis of acute promyelocytic leukemia (APL), with t(15;17)(q23;q21.1) in all metaphases. Induction therapy commenced with daunorubicin, cytarabine (Ara-C), and all-trans retinoic acid (ATRA), and complete remission was documented 5 weeks later. PML-RARA fusion transcripts were still detected by reverse transcription polymerase chain reaction. He is now referred to you for consideration of postremission therapy in the setting of high-risk acute promyelocytic leukemia in first remission.

Acute Promyelocytic Leukemia Suggesting a Diagnosis of Myelodysplastic Syndrome: a Diagnostic Dilemma.

Although acute promyelocytic leukemia displays a wide spectrum of morphological variants, the classic morphological variants are defined as hypergranular variants and the hypogranular or microgranular form. We report here for the first time a rare morphological variant of APL mimicking myelodysplastic syndrome. A 49-year-old man presented with pancytopenia. Laboratory findings were as follows: White blood cell count was 1.39 x 109/L, hemoglobin was 120 g/L, platelet count was 103 x 109/L, fibrinogen was 3.42 g/L and D-dimer was 3.14 mg/L. Peripheral blood smear revealed no blast or immature cells. Very careful inspection of bone marrow smears indicated 5% blast cells, of which 50% had hyperbasophilic cytoplasm and obvious cytoplasmic blebs mimicking megakaryoblasts or micromegakaryocytes, the others were promyelocytes with obvious bilobed nuclei and fine azurophilic granules. One or a few Auer rods could be seen in 0.5% of the blasts. Myeloperoxidase was strongly positive in the blast cells. Immunophenotyping of the BM revealed an abnormal myeloid population, which was negative for CD34 and HLA-DR. Cytogenetic analysis of the bone marrow cells revealed a 46,XY,t(15;17)(q24;q21)[3]/46,XY[4] karyotype, and the PML-RARA fusion gene was positive. The patient was diagnosed with acute promyelocytic leukemia according to WHO classification. The patient was initially diagnosed with myelodysplastic syndrome with excess blasts 2 based on cytological features. Due to the need for specific emergency treatment to avoid the risk of potentially fatal bleeding, recognition of the distinctive morphological features is vital for the diagnosis and prognosis of APL.

Acute myeloid leukemia

Diagnosis A complete blood count, which is a blood test, is one of the initial steps in the diagnosis of AML. It may reveal both an excess of white blood cells (leukocytosis) or a decrease (leukopenia), and a low red blood cell count (anemia) and low platelets (thrombocytopenia) can also be commonly seen. A blood film may show leukemic blast cells. Inclusions within the cells called Auer rods, when seen, make the diagnosis highly likely. A definitive diagnosis requires a bone marrow aspiration and biopsy.

First_Aid_Step2

A 14-year-old girl presents with prolonged bleeding after dental surgery and with menses, normal PT, normal or ↑ PTT, and ↑ bleeding time. Diagnosis? Treatment? A 60-year-old African-American man presents with bone pain. Workup for multiple myeloma might reveal? Reed-Sternberg cells. A 10-year-old boy presents with fever, weight loss, and night sweats. Exam shows an anterior mediastinal mass. Suspected diagnosis? Microcytic anemia with ↓ serum iron, ↓ total iron-binding capacity (TIBC), and normal or ↑ ferritin. Microcytic anemia with ↓ serum iron, ↓ ferritin, and ↑ TIBC. An 80-year-old man presents with fatigue, lymphadenopathy, splenomegaly, and isolated lymphocytosis. Suspected diagnosis? The lymphoma equivalent of CLL. A late, life-threatening complication of chronic myelogenous leukemia (CML). Auer rods on blood smear. AML subtype associated with DIC. Both have ↑ hematocrit and RBC mass, but polycythemia vera should have normal O2 saturation and low erythropoietin levels.

King Fahad Specialist Hospital Dammam

Hemato-oncology: acute leukemias, chronic leukemias, multiple myeloma and other plasma cells disorders, chronic myeloproliferative neoplasms, and lymphomas: Hodgkin and non-Hodgkin types Bone marrow failure syndromes: aplastic anemia, myelodysplastic syndromes and hereditary bone marrow failure syndromes. High dose chemotherapy and stem cells rescue [autologous transplantation] for: myeloma, lymphoma, acute myeloid leukemia] Allogeneic hematopoietic stem cell transplantation (HSCT) for: acute and chronic leukemias, bone marrow failure syndromes and relapsed /refractory Multiple myeloma and Lymphoma Microangiopathic hemolytic anemias Consultation Services for Hemoglobinpathy. Refractory idiopathic thrombocytopenic purpura. Hemolytic anemias. Cytopenias. Thromboembolic disorders and cancer-related thrombosis. Therapy-related thrombocytopenia including Heparin-induced thrombocytopenia.

List of ICD-9 codes 140–239: neoplasms

Multiple myeloma Lymphoid leukemia Acute lymphoblastic leukemia Chronic lymphocytic leukemia Myeloid leukemia Acute myelogenous leukemia Chronic myelogenous leukemia Monocytic leukemia Other specified leukemia Acute erythremia and erythroleukemia Chronic erythremia Megakaryocytic leukemia Leukemia of unspecified cell type

[CD2 and CD8 expression in acute promyelocytic leukemia].

A 34-year-old man was admitted to our hospital for a headache in March, 1995. The patient's hemoglobin was 7.5 g/dl, platelet count was 1.8 x 10(4)/microliter and white blood cell (WBC) count was 12,400/microliters with 99% myeloblasts. Myeloblasts were agranular or hypogranular but electron microscopy revealed microgranules in cytoplasm, and a few faggots were observed. The bone marrow was hyperplastic due to myeloblasts and chromosomal abnormality was recognized: 46, XY, t(15; 17) (q22; q12). PML-RAR alpha with intron 3 breakpoint of the PML locus, and rearrangements of the T-cell receptor beta and gamma genes were detected. These cells were positive for CD2 (63%), CD8 (47%), CD13 (87%) and CD33 (99%). Microgranular variant type of acute promyelocytic leukemia (APL) was diagnosed. Disseminated intravascular coagulation (DIC) was also present. The patient was treated with enocitabine, daunorubicin, 6-mercaptopurine, dalteparin sodium, anti-thrombin III concentrates and gabexate mesilate with prophylactic frozen transfusions of fresh plasma and platelet transfusions for 5 days, but WBC count did not decrease and DIC did not improve. The patient died of cerebral hemorrhage 7 days after diagnosis of APL. APL with CD8 expression has never been reported. We suggest that therapy should be modified in this type of APL and conclusions concerning the most appropriate therapeutic strategy will depend on the results of treatment of similar cases in the future.

[Marked bone marrow necrosis preceding acute myeloblastic leukemia in childhood].

A 3-year-old boy was transferred to our hospital because of fever, abdominal pain and severe systemic bone pain on October 16, 1989. Hematological examination showed hemoglobin 8.7 g/dl, white blood cell count 5300/microliters with 9% neutrophils and platelet count 5.5 x 10(4)/microliters. Bone marrow aspiration and biopsy revealed markedly necrotic cells. Blood chemistry showed transient elevation of CRP, serum LDH, FDP, FDP-Ddimer and fibrinogen. Tc99m pyrophosphate bone scanning showed multiple uptake spots in various bone. Although the sign of fever, abdominal pain and bone pain disappeared spontaneously after three weeks, anemia persisted. About two months later from bone marrow necrosis, abnormal cells appeared in the bone marrow. A diagnosis of AML (M3) was made and a combination chemotherapy started. This case is remarkable for elevation of acute phase protein in association with bone marrow necrosis.

[Giant platelet-like cell fragments produced from abnormal promyelocytes in acute myelogenous leukemia].

A 38-year old man was transmitted to our hospital because of his pneumonia and disconsciousness. Laboratory data showed leukocytosis (32,500/microliter), mild anemia, and decreased platelet count (6.7 x 10(4)/microliter). The bone marrow aspiration revealed the presence of 40% blastoid cells and cytogenetic study showed abnormal karyotype, 45, X, -Y, t(8; 21) (q22; q22), indicating acute myeloid leukemia (AML, M2). Furthermore, on the microscopic observation, cell fragments resembling giant platelets were observed which were positive for myeloperoxidase, and several fragments connected with abnormal promyelocytes through thin cytoplasm. These results suggested these cell fragments may be produced from abnormal promyelocytes in this case.

[Acute megakaryoblastic leukemia developing 11 years after diagnosis of essential thrombocythemia].

A 57-year-old man was diagnosed to have essential thrombocythemia (ET) in July 1977. He was doing well with continual medication of carboquone but was hospitalized because of slight unconsciousness and gait disturbance in May, 1988. His laboratory data were as follows: WBC count 81,600/microliters with 55% of blasts with cytoplasmic blebs, Hb 10.2 g/dl, and platelet count 2.6 x 10(4)/microliters. Bone marrow aspiration revealed hypercellular marrow with 72.8% blasts. Chromosomal analysis showed tetraploidy with 7p+ and 19p+. Cytochemistry of blasts showed the positivity for platelet peroxidase and CDw 41. The diagnosis of acute megakaryoblastic leukemia was made. Meningeal leukemia was also suspected by the cerebrospinal fluid data, and cytarabine was intrathecally injected. Then the percent of blasts of peripheral blood gradually decreased and the data of cerebrospinal fluid improved. However, several days later the patient became comatose probably due to cerebral bleeding, and died. In this case, two possibilities were considered (1) that a blastic transformation to acute leukemia from ET, and (2) that a secondary leukemia developed as a result of the chemotherapy, independently of ET. Since there was no evidence of myelodysplastic syndrome, it was concluded that this case represented a blastic transformation of ET.

Acute Promyelocytic Leukemia -- Evaluation

When acute promyelocytic leukemia is suspected, evaluation of peripheral blood smear and FISH for the fusion of PML/RARA should be expedited for rapid diagnosis of this time-sensitive disease. A prompt coagulopathy workup including a platelet count, prothrombin time (PT), activated partial thromboplastin time (PTT), d-dimer or fibrin split products, and fibrinogen should also be performed. Bone marrow biopsy and immunophenotyping should also be performed. Conventional karyotyping should also be performed as a part of initial workup as it detects rare molecular subtypes of acute promyelocytic leukemia and other additional coexistent cytogenetic abnormalities- t(15:17). Reverse transcriptase-polymerase chain reaction (RT-PCR) for PML-RARA RNA is also used for confirming the diagnosis of acute promyelocytic leukemia and can also be used can for monitoring minimal residual disease.

White blood cell differential

When present in significant quantities in the blood, immature granulocytes can indicate infection and inflammation, as well as myeloproliferative disease, leukemia and other conditions affecting the marrow. IGs may also be increased in steroid use and pregnancy. Chronic myeloid leukemia often presents with a high number of immature granulocytes in the peripheral blood. Abnormal promyelocytes with multiple Auer rods, called faggot cells, occur in acute promyelocytic leukemia. Blast cell Blast cells are very immature cells that are normally found in the bone marrow, where they develop into mature cells (hematopoiesis) before being released into the blood. They can be identified by their large overall size, deep blue cytoplasm, and large nucleus with fine chromatin and prominent nucleoli.

[Abnormality of blood coagulation indexes in patients with de novo acute leukemia and its clinical significance].

To explore hemorrhage risk and the clinical significance of abnormal change of prothrombin time (PT), activated partial thromboplastin time (APTT), plasma fibrinogen (FIB), plasma thrombin time (TT) and d-dimer (D-D) in de novo acute leukemia (except for APL), the different bleeding manifestations of 114 cases of de novo acute leukemia with different coagulation indexes were analyzed retrospectively. The correlation between these blood coagulation indexes and the possible correlative clinical characteristics were analysed, including age, sex, type of acute leukemia, initial white blood cell(WBC) and platelet(Plt) count, the proportion of blast cells in bone marrow and cytogenetic abnormality of patients at diagnosis. The results indicated that the incidence of abnormal blood coagulation was as high as 78.1% for de novo AL patients. These patients with 5 normal blood coagulation indexes may have mild bleeding manifestation, but the more abnormal indexes, the more severe bleeding. Both PT and D-D were sensitive indexes for diagnosis of level II bleeding. Incidence of abnormal blood coagulation significantly correlates with the proportion of blast cells in bone marrow (χ(2) = 4.184, OR = 1.021, P < 0.05) and more with D-D (P < 0.01), while age, sex, type of AL, WBC count, Plt count and abnormality of cytogenetics did not correlate with abnormal blood coagulation. It is concluded that the coagulation and fibrinolysis are abnormal in most patients with de novo acute leukemia. More abnormal indexes indicate more severe bleeding, and both PT and D-D are sensitive indexes for diagnosis of level II bleeding. Higher proportion of blast cells in bone marrow predicts higher incidence of abnormal blood clotting. Acute leukemia with elderly age, high white blood cell count and adverse cytogenetics do not predict severer abnormal blood clotting. Detection of PT, APTT, TT, FIB, and D-D may help to judge whether the patients are in a state of hypercoagulability or disseminated intravenous coagulation, which will provide experiment evidences for early intervention and medication.

International Classification of Diseases for Oncology

M9863/3 Chronic myeloid leukemia, NOS Chronic myelogenous leukemia, NOS Chronic granulocytic leukemia, NOS Chronic myelocytic leukemia, NOS M9866/3 Acute promyelocytic leukemia t(15;17)(q22;q11–12) * Acute promyelocytic leukemia, PML/RAR-alpha Acute myeloid leukemia, t(15:17(q22;q11–12) Acute promyelocytic leukemia, NOS FAB-M3 (includes all variants) Acute promyelocytic leukemia (AML with t(15;17)(q22;q12), PML-RARa and variants) M9867/3) Acute myelomonocytic leukemia Acute myelomonocytic leukemia FAB-M4 M9870/3 Basophilic leukemia or Acute basophilic leukemia M9871/3 Acute myeloid leukemia with abnormal marrow eosinophils (includes all variants) AML with inv(16)(p13q22) or t(16;16)(p13;q22), CBFb/MYH11 (FAB M4Eo) M9872/3 Acute myeloid leukemia, minimally differentiated (FAB type M0) Acute myeloblastic leukemia] M9873/3 Acute myeloid leukemia, without maturation (FAB type M1) M9874/3 Acute myeloid leukemia, with maturation (FAB M2), NOS

Low platelet count is potentially the most important contributor to severe bleeding in patients newly diagnosed with acute promyelocytic leukemia.

The objective of the current study was to provide more appropriate therapeutic strategies for reducing severe hemorrhaging by assessing the recovery of abnormal coagulation indexes in patients with acute promyelocytic leukemia (APL) during induction therapy. Retrospective analyses of 112 patients newly diagnosed with APL were performed during initial treatment. In our study, the early death rate was 5.36%. Hemorrhage was the leading cause of death during the induction period (4/6). The values of white blood cell count, lactate dehydrogenase, prothrombin time (PT), fibrinogen (Fbg), hemoglobin, and bone marrow leukemic promyelocytes were significantly different in the high-risk group compared to the low/intermediate-risk groups. There were significant differences in the white blood cell count, bone marrow leukemic promyelocytes, platelet (PLT) count, and the levels of lactate dehydrogenase, d-dimer, PT, and Fbg, as well as in FLT3-ITD mutations between patients with major bleeding and those with minor bleeding. Hemostatic variables significantly improved over time during induction therapy. The recovery times of the PLT, PT, and Fbg values were significantly slower in patients with major bleeding than in those with minor bleeding. Specifically, the PLT level in patients with major bleeding was not similar to that in the minor bleeding group until after 4 weeks of treatment. Hemorrhages were the most common cause of induction death in this study. High-risk patients were more prone to serious clinical bleeding symptoms. Patients with major bleeding had more rapid proliferation characteristics and an increased incidence of FLT3-ITD mutations compared to patients with minor bleeding. Hemostatic variables recovered significantly more slowly in patients with major bleeding than in those with minor bleeding. Active induction therapy and blood product infusion are effective in preventing severe bleeding. Our results suggested that low PLT count might be the leading cause of fatal bleeding in patients newly diagnosed with APL.

Coagulant and fibrinolytic activities in the leukemic cell lysates.

We attempted to examine procoagulant activity (PCA), X activator activity (XAA) and plasminogen activator activity (PlgAA) of various leukemic cell lysates: 17 acute myelocytic leukemias (AML), 4 acute promyelocytic leukemias (APL), 9 acute myelomonocytic leukemias (AMMoL), 7 chronic myelocytic leukemias (CML), 4 CML with blastic crisis, 7 T cell acute lymphocytic leukemias (ALL), 8 adult T cell leukemias (ATL), 8 null cell ALL, 6 B cell lymphocytic leukemias. Among those 70 cases, 4 APL, 4 AMMoL and 5 AML were associated with overt disseminated intravascular coagulation (DIC) and 5 T cell ALL, 7 ATL and 2 null cell ALL were associated with hypofibrinogenemia not adapted for DIC. The sample used was the lysate of 10(7) cells. PCA was measured by recalcification time of normal plasma with the cell lysate, XAA and PlgAA was measured by chromogenic substrate. APL and AML, especially those associated with overt DIC, had high PCA, and lymphocytic leukemia generally had low PCA in comparison with normal controls. Total PCA (PCA multiplied by cell count/microliter) was remarkably increased in DIC and mildly increased in ALL with hypofibrinogenemia. The change in XAA and total XAA (XAA multiplied by cell count/microliter) was not remarkable in any leukemia except for T cell ALL and null cell ALL with hypofibrinogenemia. PlgAA was high in lymphocytic leukemias with hypofibrinogenemia, APL and AMMoL with DIC. Total PlgAA (PlgAA multiplied by cell count/microliter) was high especially in T cell ALL and null cell ALL with hypofibrinogenemia. Thus it is probable that PCA is the most important factor causing DIC in myelogenous leukemia and that PlgAA is the most important factor causing hypofibrinogenemia in lymphocytic leukemia. The measurement of these activities in the leukemic cells is valuable in prediction and prevention of the hemostatic disorder in leukemia.

ETV6

Medical significance Inherited mutations Rare missense and other loss of function mutations in ETV6 cause thrombocytopenia 5, an autosomal dominant familial disease characterized by variable thrombocytopenia (blood platelet counts from 5% to 90% of normal), mild to modest bleeding tendencies, and bone marrow biopsy findings of abnormal appearing megakaryocytes (i.e. nuclei with fewer than the normal number of lobulations) and red cell macrocytosis. Thrombocytopenia 5 is associated with an increased incidence of developing hematological (e.g. chronic myelomonocytic leukemia, acute myelocytic leukemia, B cell acute lymphoblastic leukemia, mixed phenotype acute leukemia, Myelodysplastic syndrome, and multiple myeloma) and non-hematological (e.g. skin and colon) cancers as well as non-malignant diseases such as refractory anemia myopathies, and gastroesophageal reflux disease.

Biphenotypic acute leukaemia

Before 2008, the diagnosis of BAL was based on a score system proposed by the European Group for the Immunological Classification of Leukemias (EGIL) which could differentiate from other kinds of acute leukemia. The table shows this method. If the score of only one lineage is higher than 2, the acute leukemia could be acute myeloid leukemia (AML) or acute lymphoblastic leukemia (ALL). According to the original EGIL scoring system BAL is defined when scores are over two points for both myeloid and T- or B- lymphoid lineages. In 2008, WHO established a new and strict criteria standard for diagnosis of BAL. The presence of specific T-lymphoid antigens, cytoplasmic CD3 (cCD3), MPO and CD 19 became the most important standard for recognizing the lineage. Other B-lineage markers (CD22, CD79a, CD 10) and monocytic markers are also needed. Table 2 shows the method.

Hyperfibrinolysis Is an Important Cause of Early Hemorrhage in Patients with Acute Promyelocytic Leukemia.

BACKGROUND The objective of the current study was to guide the early clinical treatment strategies by assessing the recovery of abnormal coagulation in acute promyelocytic leukemia (APL) patients during induction therapy. MATERIAL AND METHODS Retrospective analysis was performed in 112 newly-diagnosed patients with APL during induction treatment. RESULTS The early death (ED) rate in our study was 5.36% and the main cause was fetal hemorrhage. The presence of bleeding symptoms was significantly correlated with low platelet and fibrinogen levels. The values of white blood cell (WBC), lactate dehydrogenase (LDH), prothrombin time (PT), fibrinogen, and bone marrow leukemic promyelocyte in the high-risk group were significantly different from those in the low/intermediate-risk groups. Coagulation variables significantly improved after dual induction therapy. No significant difference was found in changes of platelet (PLT), prothrombin time (PT), activated partial thromboplastin time (APTT), D-dimers, and fibrinogen among different risk groups after induction therapy. D-dimer levels were initially high and remained well above normal after 4 weeks of induction therapy. CONCLUSIONS Aggressive prophylactic transfusion to maintain high platelet and fibrinogen transfusion thresholds could reduce hemorrhage in APL patients. Immediately starting induction therapy effectively alleviated coagulopathy in APL patients. Hyperfibrinolysis was a more important event in the APL hemorrhagic diathesis.

Leukemoid reaction

Diagnosis Conventionally, a leukocytosis exceeding 50,000 WBC/mm3 with a significant increase in early neutrophil precursors is referred to as a leukemoid reaction. The peripheral blood smear may show myelocytes, metamyelocytes, promyelocytes, and rarely myeloblasts; however, there is a mixture of early mature neutrophil precursors, in contrast to the immature forms typically seen in acute leukemia. Serum leukocyte alkaline phosphatase is normal or elevated in leukemoid reaction, but is depressed in chronic myelogenous leukemia. The bone marrow in a leukemoid reaction, if examined, may be hypercellular but is otherwise typically unremarkable.

Clonal hypereosinophilia

PCM1-JAK2 gene positive patients present with features of myeloid neoplasms, lymphoid neoplasms, or features of both types of neoplasms. Most commonly, the present with features of myeloid neoplasms with 50–70% of cases associated with eosinophilia and/or bone marrow fibrosis Their disease usually progresses rapidly from a chronic phase to an acute blast cell phase resembling chronic myelogenous leukemia's conversion form chronic to acute phases. Rarely, the acute phase of PCM1-JAK2 gene positive disease resembles a lymoblastic leukemia. PCM1-JAK2-induced hematological malignances are rare and relatively newly discovered. The disease is aggressive and therefore has been treated aggressively with chemotherapy followed by bone marrow transplantation. However, of 6 patients treated with a tyrosine kinase inhibitor, ruxolitinib, 5 experienced complete remissions and have survived for at least 30 months. One patient relapsed after 18 months ruxolitinib therapy and required Hematopoietic

[Association of acute leukemia with disseminated intravascular coagulation in adults. Analysis of 14 cases].

The association of acute leukemia (AL) and disseminated intravascular coagulation (DIC) in 89 patients with a de novo diagnosis of AL made in our center during the last 8 years was retrospectively evaluated. DIC was demonstrated in 14 patients (15.7%) (7 AML-M3, 1 AML-M3, 1 AML-M2, 1 AML-M4, 2 AML-M5, and 2 ALL-L1). In 5 of them ICD was diagnosed after the beginning of chemotherapy. The factors predisposing to the development of DIC were: 1) the type of AL (p less than 0.01), as 70% of AML-M3 had DIC; 2) the intensity of granulation in leukemia cells (p less than 0.004); 3) the presence of Auer's rods and/or splinters in these cells, and 4) the presence of hemorrhagic diathesis (p less than 0.007). Eight of the 14 patients with DIC received heparin at a prophylactic dosage. No significant differences in the clinical course were in the group of patients with DIC who received heparin and in those who did not, excepting that in the former the platelet requirements were higher (p less than 0.005). Mortality rate during the first month was higher in the group of AL with DIC than in AL without DIC (p less than 0.025). Long term mortality was similar in both groups. The control of hemostasis is fundamental in AL, even in those patients without DIC at the time of diagnosis. The administration of blood derivatives has a high priority in AL with DIC. The role of heparin is still controversial.

Benzene

Health effects Benzene is classified as a carcinogen, which increases the risk of cancer and other illnesses, and is also a notorious cause of bone marrow failure. Substantial quantities of epidemiologic, clinical, and laboratory data link benzene to aplastic anemia, acute leukemia, bone marrow abnormalities and cardiovascular disease. The specific hematologic malignancies that benzene is associated with include: acute myeloid leukemia (AML), aplastic anemia, myelodysplastic syndrome (MDS), acute lymphoblastic leukemia (ALL), and chronic myeloid leukemia (CML).

[Monocytoid B-lymphocytic leukemia].

The recently described monocytoid B-cell lymphoma is a low-grade lymphoma presenting most frequently in elderly women and commonly associated with autoimmune diseases. Leukaemic expression of this disease has been reported in advanced stages. A case of monocytoid lymphocytosis without lymph node enlargement is presented herein. A 60-year old woman complaining of easy bruises was found to have a 2-cm splenomegaly. Her laboratory data included the following: haemoglobin, 125 g/L; haematocrit, 0.35 L/L; white cell count, 29 x 10(9)/L with 32% PMN, 3% stabs, 2% myelocytes, 1% metamyelocytes, 30% lymphocytes and 32% atypical mononucleated cells showing wide, pale cytoplasm neatly contoured and oval nucleus with monocytoid features. The basal coagulation study showed prothrombin 50%, APTT 40 seconds, fibrinogen 68 mg/dL and FDP between 80 and 160 ng/dL. Splenomegaly without lymph-node enlargement was found on CT scan. The bone-marrow biopsy showed a 68% monocytoid lymphocytic infiltration, acid-phosphatase positive and tartrate-sensitive, without fibrosis. Bone-marrow and peripheral immunophenotype showed those cells to be CD22, CD 19 and CD11 positive, while T and CD25 markers were absent. The patient was treated with alpha-2b interferon at a dose of 3MU three times a week for 6 months, with general improvement and regression of the leukaemic expression. Eleven months after diagnosis she died of a central nervous system haemorrhage. The morphological, immunological and cytochemical features of the monocytoid lymphocytes in this case are commented, along with their variable behaviour. A review of the literature is also carried out, attention being laid on the onset and the response to therapy of B-cell monocytoid lymphomas as the singularity of this case lies on its exclusively leukaemic onset. It is concluded that an interrelationship between monocytoid B-lymphocytic leukaemia and B-cell monocytoid lymphoma might possibly exist, such as that between chronic lymphocytic leukaemia and diffuse lymphocytic lymphoma.

Monocytopenia

Monocytopenia is a form of leukopenia associated with a deficiency of monocytes. It has been proposed as a measure to predict neutropenia, though some research indicates that it is less effective than lymphopenia. Causes The causes of monocytopenia include: acute infections, stress, treatment with glucocorticoids, aplastic anemia, hairy cell leukemia, acute myeloid leukemia, treatment with myelotoxic drugs and genetic syndromes, as for example MonoMAC syndrome. Diagnosis - Blood Test (CBC) (Normal range of Monocytes: 1-10%) (Normal range in males: 0.2-0.8 x 10 3 /microliter) - Blood test checking for monocypenia (Abnormal ranges: <1%) (Abnormal range in males: <0.2 10 3 /microliter) Treatment References External links Monocyte and granulocyte disorders

[Disseminated intravascular coagulation (D.I.C.) and fibrinolysis in patients with acute leukemia (author's transl)].

Biological symptoms of D.I.C. were investigated in 43 patients with acute leukemia. Ten of them were found to be positive either at the onset or at the relapse of the disease and in some cases D.I.C. was triggered by chemotherapy. Among the ten positive cases 3 patients had an acute promyelocytic leukemia, 4 had an acute lymphoblastic leukemia, 2 a myeloblastic and 1 a monoblastic leukemia. D.I.C. was found either in patients with an hypercellular form of the disease or in patients with a normal or low white cell count. Symptoms of D.I.C. in acute leukemia must be systematicaly sought at the onset and during treatment by chemotherapy and treated with heparin and platelet transfusions as it is now admitted for acute promyelocytic leukemia.

PDGFRB

Similar to PDGFRB-ETV6 translocations, these translocations are generally in-frame and encode for fusion proteins with their PDGFRB-derived tyrosine kinase being continuously active and responsible for causing the potentially malignant growth of its myeloid and/or lymphoid harboring cells. Patients are usually middle-aged men. They commonly present with anemia, eosinophilia, monocytosis, and splenomegaly and have their disease classified as chronic myelomonocytic leukemia, atypical chronic myelomonocytic leukemia, juvenile myelomonocytic leukemia, myelodysplastic syndrome, acute myelogenous leukemia, acute lymphoblastic leukemia, or T lymphoblastic lymphoma. Diagnosis relies on cytogenetic analyses to detect breakpoints in the long arm of chromosome 5 by Fluorescence in situ hybridization. These patients usually respond well to imatinib therapy.

[Therapy of disseminated intravascular coagulation in acute promyelocytic leukemias. Apropos of 19 cases].

To evaluate our experience in the treatment of disseminated intravascular coagulation (DIC) associated to AML-M3 with substitution therapy with or without heparin. The clinical records of 19 patients diagnosed of AML-M3 in the Hospital Juan Canalejo, in La Coruña, between 1986 and 1991 were revised. DIC was defined by abnormalities in one or more of the following: prothrombin time, activated partial thromboplastic time, thrombin time, fibrinogenaemia, fibrin/fibrinogen degradation products, D-dimers. The treatment given to 13 patients was only substitutive including platelets, fresh-frozen plasma, cryoprecipitates and antithrombin III. Low-dose sodium heparin (Kabi) in continuous intravenous infusion was associated to the treatment of the remaining 6 patients. With regard to the leukaemic therapy, most of the patients received daunorubicin, 2 mg/kg/day x 5 days, and AraC, 100 mg/Kg/day x 7 days. All patients showed haemorrhagic symptoms at diagnosis, and laboratory data of DIC were present in 11 cases (57%). The mean duration of DIC was 6.5 days for the patients receiving substitutive treatment and 9.5 days for those given heparin. Complete remission (CR) of the leukaemia was attained in 12 cases (63%); of the patients treated with heparin, 50% achieved CR whereas 75% of those receiving substitute therapy attained CR. The 24-months survival rate was 30% for the patients treated with substitutive therapy and 16% for those treated with heparin. The incidence of death associated to haemorrhage during induction therapy was 30% for the substitutive treatment group and 50% for the heparin group. Shorter duration of DIC, lesser rate of early death, higher CR rate and longer 2-year survival were found in those AML-M3 patients not receiving heparin.

Leukemia

The exact cause of leukemia is unknown. A combination of genetic factors and environmental (non-inherited) factors are believed to play a role. Risk factors include smoking, ionizing radiation, some chemicals (such as benzene), prior chemotherapy, and Down syndrome. People with a family history of leukemia are also at higher risk. There are four main types of leukemia—acute lymphoblastic leukemia (ALL), acute myeloid leukemia (AML), chronic lymphocytic leukemia (CLL) and chronic myeloid leukemia (CML)—as well as a number of less common types. Leukemias and lymphomas both belong to a broader group of tumors that affect the blood, bone marrow, and lymphoid system, known as tumors of the hematopoietic and lymphoid tissues.

Acute promyelocytic leukemia with (15;17) translocation and chromosome no. 11 deletion (q23).

The patient, a 76-year-old man, was referred with fever, large ecchymotic lesions and ulcerative laryngitis. Blood counts showed a hemoglobin of 11 g/100 ml, hematocrit of 31%, red blood cell count of 3.5 X 10(12)/1, white blood cell count of 6.8 X 10(9)/1 and platelet count of 16.0 X 10(9)/1. The differential count showed 17% neutrophils, 4% lymphocytes, 40% promyelocytes and 39% myeloblasts. The sternal marrow sample showed a marked hypercellularity. Of the cells, 80-85% were hypergranular promyelocytes, some of them showing bundles of Auer rods. No granulocytic maturation was observed. A few erythroblasts were present. A disseminated intravascular coagulation was observed (fibrinogen 0.85 g/l, factor V 18%, fibrin degradation products 640 mg/l). The serum creatinin was at 217 micromol/1 and the urea at 16.8 mmol/1. The treatment (daunorubicin, heparin, platelet transfusion) was unsuccessful and the patient died three days after entering hospital. The bone marrow karyotype by direct examination showed only normal metaphases (32 photographed). All the metaphases from the unstimulated blood 48-h culture (25 photographed) were clonal, showing the pattern 47,XY,del(11) (q23),t(15;17) (q24? q22?), +mar. The marker was '16 like' in size but its origin could not be determined (Figs. 1 and 2).

Acute promyelocytic leukemia

Diagnosis Acute promyelocytic leukemia can be distinguished from other types of AML based on microscopic examination of the blood film or a bone marrow aspirate or biopsy as well as finding the characteristic rearrangement. The presence of promyelocytes containing multiple Auer rods (termed faggot cells) on the peripheral blood smear is highly suggestive of acute promyelocytic leukemia. Definitive diagnosis requires testing for the PML/RARA fusion gene. This may be done by polymerase chain reaction (PCR), fluorescent in situ hybridization (FISH), or conventional cytogenetics of peripheral blood or bone marrow. This mutation involves a translocation of the long arm of chromosomes 15 and 17. On rare occasions, a cryptic translocation may occur which cannot be detected by cytogenetic testing; on these occasions PCR testing is essential to confirm the diagnosis. Treatment Initial treatment

Biological symptoms of D.I.C. were investigated in 43 patients with acute leukemia. Ten of them were found to be positive either at the onset or at the relapse of the disease and in some cases D.I.C. was triggered by chemotherapy. Among the ten positive cases 3 patients had an acute promyelocytic leukemia, 4 had an acute lymphoblastic leukemia, 2 a myeloblastic and 1 a monoblastic leukemia. D.I.C. was found either in patients with an hypercellular form of the disease or in patients with a normal or low white cell count. Symptoms of D.I.C. in acute leukemia must be systematically sought at the onset and during treatment by chemotherapy and treated with heparin and platelet transfusions as it is now admitted for acute promyelocytic leukemia.


From the symptoms reported, tingling in the anterior thigh, scrotum and dysesthesia in the feet, there seems to be a vertebral involvement at L2-3 and sacral roots, so the correct thing to do would be to perform a radiological examination without mobilizing the patient. But it is necessary to mobilize him to explore him, obviously, en bloc. The cervical spine seems to be undamaged because the patient mobilizes both arms adequately, without paresthesia.

From the symptoms reported, tingling in the anterior thigh, scrotum and dysesthesia in the feet, there seems to be a vertebral involvement at L2-3 and sacral roots, so [HIDDEN] would be to perform a radiological examination without mobilizing the patient. But it is necessary to mobilize him to explore him, obviously, en bloc. The cervical spine seems to be undamaged because the patient mobilizes both arms adequately, without paresthesia.

A 30-year-old man suffers fall from a standing height of 2 meters. On arrival at the ER, he is cyo, catheterized with clear urine. He shows swelling of the left heel, as well as mild tingling in the anterior aspect of the right thigh and scrotum, and dysesthesia in both feet. The patient says goodbye to the stretcher-bearer with a handshake, with a simultaneous pat on the shoulder. Rotation of the MMII on the stretcher grasped by the ankles is painless and compression of the pelvis is asymptomatic. Next:

We will sit him on the stretcher to be able to explore the cervical spine.

We will refer to Rx to perform lower extremities study.

Palpate the abdomen and rotate it en bloc to palpate the thoracolumbar spine.

Place a cervical collar and refer to radiology for cervical spine and lower extremity imaging.

May–Thurner syndrome

Signs and symptoms Because of its similarities to Deep Vein Thrombosis (DVT), May-Thurner’s syndrome is rarely diagnosed amongst the general population. In this condition, the right iliac artery sequesters and compresses the left common iliac vein against the lumbar section of the spine, resulting in swelling of the legs and ankles, pain, tingling, and/or numbness in the legs and feet. The pain is often presented as dull, and may progress up and down the leg depending on the patient. Lower extremities may feel warm to the touch and swelling may maintain or dissipate throughout the day.

GALS screen

Now ask the patient to do the following noting any painful, restricted or asymmetrical movements: Test rotation of the thoracic and lumbar spine. Gently hold the patient's hips still and ask them to: "Turn your shoulders round as far as you can to the left, then do the same to the right." Test lateral flexion of the thoracic and lumbar spine: "Stand up straight and then slide the palm of your right hand down your thigh towards your knee, bending your shoulder down to the side." "Now do the same with your left hand down your left leg." "Bend your left ear down towards your left shoulder and then your right ear down towards your right shoulder" to test for pain free cervical spine lateral flexion. Now test for stiffness or pain flexing or extending the cervical spine: "bend your neck forwards to try to touch your chin against your chest." "bend your neck back to lift your chin."

Casualty lifting

The first in place is the chief. The stretcher is slid besides the casualty, the pole against the thigh of the chief. Then, the third team member takes place. Once the both extremities of the pole are blocked, the other team members can step over the casualty (one by one, holding the others' shoulder to avoid falling) without any risk of rocking for the stretcher. On the order of the chief, the casualty is lifted and translated on the stretcher. This method can be performed with only three first responders. In this case, the chief plays the role of the first team member; blocking the pole with his/her ankle, and putting one hand under the neck, the other one under the back, between the shoulder blades. Only the team member at the hips steps over the stretcher. Rolling methods (log roll)

Cervical Intradural Disc Herniation Causing Progressive Quadriparesis After Spinal Manipulation Therapy: A Case Report and Literature Review.

Cervical intradural disc herniation (IDH) is an extremely rare condition, comprising only 0.27% of all disc herniations. Three percent of IDHs occur in the cervical, 5% in the thoracic, and over 92% in the lumbar spinal canal. There have been a total of 31 cervical IDHs reported in the literature. The pathogenesis and imaging characteristics of IDH are not fully understood. A preoperative diagnosis is key to facilitating prompt intradural exploration in patients with ambivalent findings, as well as in preventing reoperation. The purpose of reporting our case is to remind clinicians to consider the possibility of cervical IDH during spinal manipulation therapy in patient with chronic neck pain.The patient signed informed consent for publication of this case report and any accompanying image. The ethical approval of this study was waived by the ethics committee of Chonbuk National University Hospital, because this study was case report and the number of patients was <3.A 32-year-old man was transferred our emergency department with progressive quadriparesis. He had no history of trauma, but had received physical therapy with spinal manipulation for chronic neck pain over the course of a month. The day prior, he had noticed neck pain and tingling in the bilateral upper and lower extremities during the manipulation procedure. The following day, he presented with bilateral weakness of all 4 extremities, which rendered him unable to walk. Neurological examination demonstrated a positive Hoffmann sign and ankle clonus bilaterally, hypoesthesia below the C5 dermatome, 3/5 strength in the bilateral upper extremities, and 2/5 strength in the lower extremities. This motor weakness was progressive, and he further complained of voiding difficulty.Urgent magnetic resonance imaging (MRI) of the cervical spine revealed large, central disc herniations at C4-C5 and C5-C6 that caused severe spinal cord compression and surrounding edema. We performed C4-C5-C6 anterior cervical discectomy and fusion.The patient's limb weakness improved rapidly within 1 day postoperatively, and he was discharged 4 weeks later. At his 12-month follow-up, the patient had recovered nearly full muscle power.We presented an extremely rare case of cervical IDH causing progressive quadriparesis after excessive spinal manipulation therapy. The presence of a "halo" and "Y-sign" were useful MRI markers for cervical IDH in this case.

InternalMed_Harrison

The straight leg–raising (SLR) maneuver is a simple bedside test for nerve root disease. With the patient supine, passive flexion of the extended leg at the hip stretches the L5 and S1 nerve roots and 113 CHAPTER 22 Back and Neck Pain 4th Lumbar vertebral body 5th Lumbar vertebral body 4th Lumbar pedicle L4 root Protruded L4-L5 disk L5 Root S1 Root S2 Root Protruded L5-S1 disk FIguRE 22-3 Compression of L5 and S1 roots by herniated disks. (From AH Ropper, MA Samuels: Adams and Victor’s Principles of Neurology, 9th ed. New York, McGraw-Hill, 2009; with permission.) the sciatic nerve. Passive dorsiflexion of the foot during the maneuver adds to the stretch. In healthy individuals, flexion to at least 80° is normally possible without causing pain, although a tight, stretching sensation in the hamstring muscles is common. The SLR test is positive if the maneuver reproduces the patient’s usual back or limb pain. Eliciting the SLR sign in both the supine and sitting positions can help

Central cord syndrome in a high school wrestler: a case report.

To alert athletic trainers to the importance of recognizing the signs and symptoms of central cord syndrome. A 15-year-old high school wrestler was found lying supine on the mat after sustaining a hyperextension injury to his neck while drilling during practice, complaining of numbness, tingling, and a burning sensation in all 4 extremities. Touching the extremities elicited an extreme burning sensation. After in-line stabilization of the cervical spine was performed, palpation of the spinous processes elicited tenderness and an increase in pain. Six weeks before the injury, the athlete had experienced forced lateral flexion of the cervical spine during a match, resulting in an episode of bilateral numbness and burning in his arms. On evaluation by the athletic trainer, symptoms were limited to the right hand, and brachial plexus neurapraxia was diagnosed. Central cord syndrome, brachial plexus injury, cervical spine injury, burning hands syndrome, Brown-Séquard syndrome, anterior cord syndrome. Upon assessment, the athlete's cervical spine was immobilized until emergency medical services arrived and applied a cervical collar. Radiographs taken at the hospital revealed a congenital fusion of C6-7. Magnetic resonance imaging and computed tomography showed evidence of stenosis, a herniated disc at C3-4, and a central cord injury. He was admitted to the hospital for observation and was placed on a corticosteroid protocol. At approximately 1 week after the injury, the athlete underwent a cervical decompression and fusion at C3-4. Subsequently, he underwent extensive rehabilitation and has had some persistent neck stiffness. The athlete is no longer allowed to participate in contact sports as a result of the presence of stenosis at multiple levels. Central cord syndrome is typically seen in an older population with cervical spondylosis and rarely occurs in young adolescents. However, this athlete sustained 2 central cord injuries, 1 mild and 1 severe, in less than 6 weeks' time. The original injury sustained by the wrestler was thought to be a brachial plexus injury but, in fact, was a mild central cord injury. Central cord syndrome was not suspected in the original injury because the athlete's complaint was of unilateral numbness. With the second injury, the central cord injury was more severe. Proper recognition, assessment, and handling of this situation were crucial in providing optimal care to this athlete.

Neurology_Adams

The pain of herniated intervertebral disc varies in severity from a mild aching discomfort to severe knife-like stabs that radiate the length of the leg and are superimposed on a constant intense ache. Sciatic pain is perceived by the patient as originating deep in the buttock and radiating to the posterolateral thigh; it may progress to the calf and ankle—to the medial malleolus (L4), lateral malleolus (L5), or heel (S1). Distal radiation to the foot is infrequent and should raise concern of an alternative process. Abortive forms of sciatica may produce aching discomfort only in the lower buttock or proximal thigh and occasionally only in the lower hamstring or upper calf. With the most severe pain, the patient is forced to stay in bed, avoiding the slightest movement; a cough, sneeze, or strain is intolerable. The most comfortable position is lying on the back with legs flexed at the knees and hips and the shoulders raised on pillows to obliterate the lumbar lordosis. For some

Adson's sign

There is minimal evidence of interexaminer reliability. Thoracic outlet obstruction may be caused by a number of abnormalities, including degenerative or bony disorders, trauma to the cervical spine, fibromuscular bands, vascular abnormalities, and spasm of the anterior scalene muscle. Symptoms are due to compression of the brachial plexus and subclavian vasculature, and consist of complaints ranging from diffuse arm pain to a sensation of arm fatigue, frequently aggravated by carrying anything in the ipsilateral hand or doing overhead work such as window cleaning. Process The patient is placed in a sitting position, hands resting on thighs. The examiner palpates radial pulse on side being tested Patient actively rotates head to ipsilateral side being tested while the examiner laterally rotates and extends the patient's shoulder Patient takes a deep breath and is instructed to hold it

InternalMed_Harrison

PART 2 Cardinal Manifestations and Presentation of Diseases FIguRE 22-1 Vertebral anatomy. (From A Gauthier Cornuelle, DH Gronefeld: Radiographic Anatomy Positioning. New York, McGraw-Hill, 1998; with permission.) Radicular pain is typically sharp and radiates from the low back to a leg within the territory of a nerve root (see “Lumbar Disk Disease,” below). Coughing, sneezing, or voluntary contraction of abdominal muscles (lifting heavy objects or straining at stool) may elicit the radiating pain. The pain may increase in postures that stretch the nerves and nerve roots. Sitting with the leg outstretched places traction on the sciatic nerve and L5 and S1 roots because the nerve passes posterior to the hip. The femoral nerve (L2, L3, and L4 roots) passes anterior to the hip and is not stretched by sitting. FIguRE 22-2 Spinal column. (From A Gauthier Cornuelle, DH Gronefeld: Radiographic Anatomy Positioning. New York, McGraw-Hill, 1998; with permission.)

Cervical spine motion in manual versus Jackson table turning methods in a cadaveric global instability model.

A study of spine biomechanics in a cadaver model. To quantify motion in multiple axes created by transfer methods from stretcher to operating table in the prone position in a cervical global instability model. Patients with an unstable cervical spine remain at high risk for further secondary injury until their spine is adequately surgically stabilized. Previous studies have revealed that collars have significant, but limited benefit in preventing cervical motion when manually transferring patients. The literature proposes multiple methods of patient transfer, although no one method has been universally adopted. To date, no study has effectively evaluated the relationship between spine motion and various patient transfer methods to an operating room table for prone positioning. A global instability was surgically created at C5-6 in 4 fresh cadavers with no history of spine pathology. All cadavers were tested both with and without a rigid cervical collar in the intact and unstable state. Three headrest permutations were evaluated Mayfield (SM USA Inc), Prone View (Dupaco, Oceanside, CA), and Foam Pillow (OSI, Union City, CA). A trained group of medical staff performed each of 2 transfer methods: the "manual" and the "Jackson table" transfer. The manual technique entailed performing a standard rotation of the supine patient on a stretcher to the prone position on the operating room table with in-line manual cervical stabilization. The "Jackson" technique involved sliding the supine patient to the Jackson table (OSI, Union City, CA) with manual in-line cervical stabilization, securing them to the table, then initiating the table's lock and turn mechanism and rotating them into a prone position. An electromagnetic tracking device captured angular motion between the C5 and C6 vertebral segments. Repeated measures statistical analysis was performed to evaluate the following conditions: collar use (2 levels), headrest (3 levels), and turning technique (2 levels). For all measures, there was significantly more cervical spine motion during manual prone positioning compared with using the Jackson table. The use of a collar provided a slight reduction in motion in all the planes of movement; however, this was only significantly different from the no collar condition in axial rotation. Differences in gross motion between the headrest type were observed in lateral bending (Foam Pillow<Prone View, P=0.045), medial lateral translation (Foam Pillow<Mayfield, P=0.032), and anterior posterior translation (Prone View<Mayfield, P=0.030). The data suggest that the manual transfer technique produces 2 to 3 times more cervical spine angular motion than the Jackson table method of transfer. The use of a collar provides significant benefit in limiting spine motion that is only observed in axial rotation. Choice of headrest does have a significant effect on the amount of motion allowed during turning, with the Foam Pillow and Prone View generally providing more effective stabilization compared with the Mayfield.

InternalMed_Harrison

Spondylotic myelopathy is one of the most common causes of chronic cord compression and of gait difficulty in the elderly. Neck and shoulder pain with stiffness are early symptoms; impingement of bone and soft tissue overgrowth on nerve roots results in radicular arm pain, most often in a C5 or C6 distribution. Compression of the cervical cord, which occurs in fewer than one-third of cases, produces a slowly progressive spastic paraparesis, at times asymmetric and often accompanied by paresthesias in the feet and hands. Vibratory sense is diminished in the legs, there is a Romberg sign, and occasionally there is a sensory level for vibration or pinprick on the upper thorax. In some cases, coughing or straining produces leg weakness or radiating arm or shoulder pain. Dermatomal sensory loss in the arms, atrophy of intrinsic hand muscles, increased deep-tendon reflexes in the legs, and extensor plantar responses are common. Urinary urgency or incontinence occurs in advanced cases, but

Unnecessary Cervical Epidural Injection in An Octogenarian.

Epidural spine injections (ESI) have no documented long-term efficacy. Furthermore, cervical ESI uniquely risk intramedullary injections with resultant neurological deficits (e.g. monoplegia to quadriplegia), and intravascular vertebral injections (e.g. which potentially contribute to stroke, brain stem infarction). A patient in his mid-eighties presented with 1 year's duration of neck pain without any accompanying numbness, tingling or weakness in the upper or lower extremities. He had no radiculopathy, myelopathy, or neurological deficit. Two years earlier, the patient sustained a myocardial infarction (MI), requiring over 5 stents and a defibrillator. At the time of presentation, he was still on a baby ASA (81 mg/day), on anti-hypertensives, and cholesterol-lowering medications. His non-contrast cervical CT scan (patient had a pacemaker/defibrillator and could not have an MR) from the summer of 2018 showed no significant spinal cord or nerve root compression at any level. Nevertheless, he was subjected to two cervical epidural injections in the early fall; his baby ASA was stopped 5 days prior to each of these injections. Notably, this placed him at increased risk of MI and/or stroke. When he was seen by neurosurgery, without any neurological deficit or significant cervical radiographic findings, he was referred back to neurology for continued conservative management. Patients are increasingly subjected to epidural cervical spinal injections that have no documented long-term efficacy, and expose them to significant risks/complications. This 80+ year-old patient, without a neurological deficit or significant cervical CT-documented pathology, underwent 2 cervical ESI that unnecessarily exposed him to potential cardiac-stent related thrombosis (e.g. stopping ASA for 5 days-a bona-fide requirement for ESI to avoid acute epidural hematomas).

InternalMed_Harrison

The most common initial symptom in patients with spinal cord compression is localized back pain and tenderness due to involvement of vertebrae by tumor. Pain is usually present for days or months before other neurologic findings appear. It is exacerbated by movement and by coughing or sneezing. It can be differentiated from the pain of disk disease by the fact that it worsens when the patient is supine. Radicular pain is less common than localized back pain and usually develops later. Radicular pain in the cervical or lumbosacral areas may be unilateral or bilateral. Radicular pain from the thoracic roots is often bilateral and is described by patients as a feeling of tight, band-like constriction around the thorax and abdomen. Typical cervical radicular pain radiates down the arm; in the lumbar region, the radiation is down the legs. Lhermitte’s sign, a tingling or electric sensation down the back and upper and lower limbs upon flexing or extending the neck, may be an early sign of

Psoas major muscle

For example, when one performs a sit-up that brings the torso (including the lower back) away from the ground and towards the front of the leg, the hip flexors (including the iliopsoas) will flex the spine upon the pelvis. Owing to the frontal attachment on the vertebrae, rotation of the spine will stretch the psoas. Clinical significance Tightness of the psoas can result in spasms or lower-back pain by compressing the lumbar discs. A hypertonic and inflamed psoas can lead to irritation and entrapment of the ilioinguinal and the iliohypogastric nerves, resulting in a sensation of heat or water running down the front of the thigh. Psoas can be palpated with active flexion of the hip. A positive psoas contracture test and pain with palpation reported by the patient indicate clinical significance. Care should be taken around the abdominal organs, especially the colon when palpating deeply.

Glossary of medicine

Back pain – is pain felt in the back. It is divided into neck pain (cervical), middle back pain (thoracic), lower back pain (lumbar) or coccydynia (tailbone or sacral pain) based on the segment affected. The lumbar area is the most common area for pain, as it supports most of the weight in the upper body. Episodes of back pain may be acute, sub-acute, or chronic depending on the duration. The pain may be characterized as a dull ache, shooting or piercing pain, or a burning sensation. Discomfort can radiate into the arms and hands as well as the legs or feet, and may include numbness, or weakness in the legs and arms. Basal Anatomy: In the direction of the base. Antonym apical. Physiology: Lowest sustained level or minimum level required, as in basal metabolic rate.

Cervical myelopathy due to a solitary osteochondroma: a case report.

Osteochondroma is the most common benign bone tumor. However, the incidence of osteochondroma in the spine is reported to be very rare. This report presents the case of a 57-year-old man who suffered from osteochondroma of the cervical spine. He had bilateral lower extremity pain for 3 years, developing pain of right upper extremity and gait disturbance. Plain radiographic images and computed tomography scans showed bony lesion in right C6/7 foramen and C6 lamina. Magnetic resonance images of whole spine showed severe compression of spinal cord at the C6/7 and spinal canal stenosis at the L3/4 level. First, we performed a surgery of the cervical spine, and removed the tumor covered with the cartilaginous cap. The pathological diagnosis of the tumor was osteochodroma. After the surgery, the symptoms on his right upper extremity improved smoothly. Because the bilateral lower extremity pain remained, a L3/4 partial laminectomy was performed 1 month later, and the symptom improved. At 1 year after his primary operation, we could not find a recurrence of the tumor. It is very important to perform a complete en bloc resection of the tumor (especially cartilaginous cap) to prevent the recurrence.

[What is your diagnosis? Aneurysm of the abdominal aorta destroying the lumbar spine].

A 58 year old woman suffering from lumbalgia over 3 years is admitted for bilateral cruralgia. Ten years before, she was treated for cervix carcinoma by hysterectomy, pelvic and lumbo-aortic radiotherapy and chemotherapy. On admission, physical examination reveals L2 level hypoesthesia and abolition of deep tendon reflexes. Plain films and CT scan show a lysis of L3, L4 and L5 vertebral bodies and a cuneiform appearance of vertebral body of L2 due to a large abdominal aortic aneurysm.

Accessory Clavicular Sternocleidomastoid Causing Torticollis in an Adult.

Sternocleidomastoid anomalies are mostly discovered in cadavers during routine dissection. Such anomalies causing torticollis are rare in adults. We report a case of accessory mid-clavicular head of the sternocleidomastoid causing torticollis in an adult. A previously healthy 27-year-old man presented with neck and shoulder pain following heavy manual work. On examination, he had mild restriction of left tilt of the head and right rotation of the neck. Palpation revealed a bipartite right sternocleidomastoid. There was no abnormal posturing of other body parts. The range of motion of the cervical spine was limited to rotation of 70° and tilt of 38° on the left side and rotation of 65°and tilt of 46° on the right side. Neck extension was 40°. The accessory sternocleidomastoid muscle belly was visible and inserted at the middle of the clavicle. Musculoskeletal ultrasound imaging confirmed the diagnosis. Radiological images of the cervical spine and electromyography were normal. Myectomy of the lateral accessory clavicular belly of the sternocleidomastoid was planned, but the patient declined this treatment. Currently, he uses oral analgesics and participates in occasional sessions of physical therapy. An accessory sternocleidomastoid can result in torticollis because it causes a physical restriction preventing the neck from tilting and rotating to the opposite side. In mild cases, the anomaly may remain undiagnosed until adulthood and can be confused with cervical dystonia and fibromatosis colli. Knowledge of the anatomy and possible variants of the sternocleidomastoid muscle is of the utmost importance to medical practitioners involved in the diagnosis and management of problems in the neck area. Variants of the sternocleidomastoid can be a concern for surgeons, physicians, and anesthetists performing interventional procedures on the neck because of possible confusion of anatomical landmarks.

Recognition of prodromal cervical spondylotic myelopathy presenting in a U.S. Veteran referred to chiropractic for acute thoracic pain: A case report.

To highlight a patient who was referred to a VA chiropractic clinic for thoracic pain and upon physical exam was found to be myelopathic, subsequently requiring surgery. A 58-year-old male attended a telephone interview with the VA chiropractic clinic for thoracic pain of 4 months duration; he denied neck pain, upper extremity symptoms or clumsiness of the feet or hands. At his in-person visit, he acknowledged frequently dropping items. The physical examination revealed signs of myelopathy including positive Hoffman's bilaterally, 3+ brisk patellar reflexes, and 5+ beats of ankle clonus bilaterally. He also had difficulty walking heel/toe. Cervical and thoracic radiographs were ordered and a referral was placed to the Physical Medicine and Rehabilitation (PM&R) Clinic for evaluation of the abnormal neurologic exam and suspicion of cervical spondylotic myelopathy (CSM). He was treated for 2 visits in the chiropractic clinic for his thoracic pain, with resolution of thoracic symptoms. No treatment was rendered to the cervical spine. The PM&R physician ordered a cervical MRI which demonstrated severe central canal stenosis and increased T2 signal within the cord at C5-C6, representing myelopathic changes. The PM&R specialist referred him to Neurosurgery which resulted in a C5-6, C6-7 anterior cervical discectomy and fusion. The importance of physical examination competency and routine thoroughness cannot be overstated. Swift identification of pathologic signs by the treating chiropractor resulted in timely imaging and surgical intervention.

Neurology_Adams

A common cause of neck, shoulder, and arm pain is disc herniation in the lower cervical region; the process is comparable to disc herniation in the lumbar region but gives rise, of course, to a different set of symptoms (Table 10-1). The problem appears most often without a clear and immediate cause, but it may develop after trauma, which may be major or minor (from sudden hyperextension of the neck, falls, diving accidents, forceful manipulations as discussed by Kristoff and Odom). The roots most commonly involved are the seventh (in 70 percent of cases) and the sixth (in 20 percent of cases); fifthand eighth-root compression makes up the remaining 10 percent (Yoss et al). The clinical diagnosis is established by a fairly discrete distribution of pain or paresthesias that corresponds to a single cervical root, reflex loss in the segment of the root, and by elicitation of exaggerated root pain with mechanical tests such as the Spurling maneuver, in which the examiner exerts downward

Upper Cervical Leiomyoma in an Adolescent Male.

Leiomyomas are benign epithelial tumors with a female preponderance usually in the uterus, gastro-intestinal tract and skin. They are well-capsulated tumors with no mitotic activity and little pleomorphism. Primary leiomyoma in the upper cervical spine region is rare and the occurrence of these lesions in young immunocompetent males is extremely rare. A 15-year-old male had swelling over the nape of the neck for 4 years with slight difficulty in neck movements for a few months. Asymptomatic 4 years back be developed a painless swelling at the nape of the neck on the right side initially the size of a pea which gradually increased size. A 10x8cm firm, on-tender and non-pulsatile swelling at the nape of the neck on the right extending from the occipital nuchal line and crossing the midline up to the posterior border of the sternomastoid. The skin over the swelling wasn't adherent and the swelling did not reduce on neck movements. Neck movements were terminally restricted with restriction on rotation towards the right. Routine X-ray's and magnetic resonance imaging were suggestive of a soft tissue mass in the inter-muscular plane on the posterior aspect more on the right side with a cystic component and causing thinning of the C2 lamina with no intraspinal extension. Biopsy was done. Findings were suggestive of a spindle cell benign tumor. Posterior en bloc excision was planned and the lesion blog with the entire capsule was excised and sent for histopathology which revealed the lesion was a leiomyoma. The patient has shown no clinical or radiological evidence of recurrence at 4 year fol-low-up. Leiomyomas in the upper cervical spine is extremely rare in adolescent immunocom-petent male.

Straight leg raise

In order to make this test more specific, the ankle can be dorsiflexed and the cervical spine flexed. This increases the stretching of the nerve root and dura. Interpretation If the patient experiences sciatic pain, and more specifically pain radiating down the leg (radiculopathy), when the straight leg is at an angle of between 30 and 70 degrees, then the test is positive and a herniated disk is a possible cause of the pain. A negative test suggests a likely different cause for back pain. A positive straight leg test reproduces radiating leg pain. If it only causes back pain, then the test is negative. Because this is often misunderstood, it is prudent to add a statement of clarification. For example, "Straight leg test is positive on the left, reproducing the patient's radiating leg symptoms." A meta-analysis reported the accuracy as: sensitivity 91% specificity 26%

[Torsion of appendix of testis and epididymis: a report of 4 cases].

We report our clinical and pathological observations on four patients with torsion of appendix testis or epididymis, and reviewed 72 cases of torsion of the appendages of intrascrotal organs collected from the Japanese literature; 35 cases of torsion of appendix testis, 36 cases of torsion of appendix epididymis and 1 case of torsion of paradidymis . Case 1 was a 10-year-old boy visiting us because of pain and swelling in his right scrotum continuing for the past ten days. His right scrotum was found to contain a hen-egg sized tender mass. The testis and epididymis could not be differentiated by palpation. The blood count disclosed 10,000 white blood cells. At operation, two appendix epididymis were found in his right scrotum. One of them was twisted 360 degrees clockwise and 10 x 8 x 5 mm in size. Case 2 was a 11-year-old boy with complaint of pain in his left lower abdomen and left scrotum for the past three days. Palpation of his left scrotal contents revealed a slightly hard testis and tender epididymis. At operation, we found his left spermatic cord to be twisted 90 degrees counterclockwise and appendix epididymis 180 degrees counterclockwise. The twisted appendix epididymis was 10 x 8 x 7 mm in size. Case 3 was a 13-year-old boy whose complaint was left scrotal pain. The upper pole of his left testis was found to be swollen and tender by palpation. The exposure of his left scrotum at operation revealed that his left epididymis was abnormally attached to his left testis and had two appendices. One of these appendix epididymis was twisted 180 degrees clockwise and measured 10 x 10 x 8 mm. Case 4 was a 19-year-old male who suffered from right testicular pain for the past seven days. He had a history of three intermittent episodes of similar right testicular pain during the past two years. His right testis was enlarged and palpated slightly hard and tender. We explored his right scrotum surgically and found the appendix testis twisted and enlarged to little-finger's head size. However, it was impossible to determine whether the rotation was clockwise or counterclockwise because the twisted appendix was too severely damaged. The preoperative diagnosis was correct in one case and three were erroneously diagnosed as having torsion of spermatic cord. All four cases were treated by surgery which relieved all patients of discomfort.

Embalming

and tibial vessels if necessary) are used. The corresponding veins are commonly also raised and utilized for drainage. Cases where more than one vessel is raised are referred to as multiple-point injection, with a reference to the number of vessels raised (i.e. a six-point injection or six-pointer). As a general rule, the more points needing to be raised, the greater the difficulty of the case. In some cases draining from a different site from injection (i.e. injecting arterial fluid into the right common carotid artery and draining from the right femoral vein) is referred to as a split (or sometimes cut) injection. In certain cases the embalmer may deem it necessary to perform a restricted cervical injection, which involves injecting the head of the deceased separately from the rest of body. This is done in cases where distention (swelling) has a greater chance of occurring. In many cases, an embalmer may select to perform what is known as a pre-injection. A pre-injection is a

Effect of 5 different cervical collars on optic nerve sheath diameter: A randomized crossover trial.

There is considerable evidence that prolonged use of cervical collars potentially cause detrimental effects including increase in optic nerve sheath diameter (ONSD) among healthy volunteers. Different types of cervical collars immobilize cervical spine variably well and may presumably differently influence the venous compression and hence the intracranial pressure. We therefore aimed to evaluate the influence of cervical spine immobilization with 5 different types of cervical collars on ONSD measured noninvasively by ultrasound on healthy volunteers. We conducted a randomized crossover trial including 60 adult healthy volunteers. Control assessment of the optic nerve sheath thickness was performed in both sagittal and transverse planes. Patient was placed supine on a transport stretcher, cervical collar was placed, and ONSD measurement was performed after 5 and 20 minutes. During the next days, the procedure was repeated with random allocation of participants and random cervical collar. Sixty healthy volunteers were included in our study. ONSD left diameter [mm] for the baseline was 3.8 [interquartile range (IQR): 3.65-3.93)] mm. Using AMBU after 5 min, ONSD was changed up to 4.505 (IQR 4.285-4.61; P < .001) mm. The largest change at 5 minutes and 20 minutes was using Philly 4.73 (IQR: 4.49-4.895; P < .001) and 4.925 (IQR: 4.65-5.06; P < .001), respectively. Necklite reported the lower change in ONSD: 3.92 (IQR: 3.795-4; P = 1.0) mm in 5 minutes and 3.995 (IQR: 3.875 - 4.1; P = 1.0) mm in 20 minutes. ONSD right diameter [mm] for the baseline was 3.8 (IQR 3.675-3.9) mm. Using AMBU after 5 minutes, ONSD was changed up to 4.5 (IQR 4.21-4.6) mm. The largest change at 5 minutes and 20 minutes was using Philly 4.705 (IQR 4.455-4.9) and 4.93 (IQR 4.645-5.075), respectively. Necklite reported the lower change in ONSD -33.9 (IQR 3.795-3.99) mm in 5 minutes and 3.995 (IQR 3.86-4.09) mm in 20 minutes. We report significant increase of ONSD from the baseline after cervical collar placement among healthy volunteers at 5 minutes and 20 minutes interval. In addition, no significant difference was noted between ONSD measurements at 5 and 20 minutes. Clinicians should take proactive steps to assess the actual need of cervical collar case by case basis. Nonetheless, when needed, Necklite moldable neck brace seems to be a reasonable option.Registration: ClinicalTrials database (www.clinicaltrials.gov, NCT03609879).

InternalMed_Harrison

in the arms, atrophy of intrinsic hand muscles, increased deep-tendon reflexes in the legs, and extensor plantar responses are common. Urinary urgency or incontinence occurs in advanced cases, but there are many alternative causes of these problems in older individuals. A tendon reflex in the arms is often diminished at some level; most often at the biceps (C5-C6). In individual cases, radicular, myelopathic, or combined signs may predominate. The diagnosis should be considered in appropriate cases of progressive cervical myelopathy, paresthesias of the feet and hands, or wasting of the hands.

The acute paediatric scrotum.

The essentials of management remain the same whatever the cause of the acutely painful scrotum. 1. History is paramount. At what time did the pain come on and with what severity? Has this changed in any way? Is there any aggravation or relief and with what is the pain associated? Are there any urinary symptoms of note? Have there been any previous episodes of pain, possibly of lesser severity? How does the patient feel? 2. Always examine the whole child. Does he look unwell or in pain? Does he have a fever? Is he tachycardic? Is his abdomen soft? 3. Stand the boy up and note the position in which he stands. Look at the scrotum. Does the skin look normal or oedematous? What is its colour? What is the lie of the testis? Remember the left usually sits a little lower than the right. Do the testes hang transversely or at an angle? Is there any swelling of the groin that might indicate a hernia? 4. Elicit the cremasteric reflex on both sides, starting with the unaffected. Examine the normal, unaffected side and determine the anatomy. Ask if you can palpate the affected side. Isolate the testis and grasp it transversely, as anteriorly as possible. Establish whether tenderness is global, superior or posterolateral. If possible, stretch the skin gently over the upper aspect and look for the 'blue dot'. 5. There are no investigations of choice save prompt surgical opinion and exploration. Remember the relative incidences of the various diagnoses.(ABSTRACT TRUNCATED AT 250 WORDS)

Neurology_Adams

Other patterns of clinical evolution In addition to the special configurations discussed further on, there are many patterns of neuromuscular involvement other than the one just described. A leg may be affected before the hands. A foot-drop with weakness and wasting of the pretibial muscles may be incorrectly attributed to peroneal nerve compression until weakness of the gastrocnemius and other muscles betray more widespread involvement of lumbosacral neurons. In our experience, this crural amyotrophy has been less frequent than the brachial-manual type. Another variant is early involvement of thoracic, abdominal, or posterior neck muscles, the last being one of the causes of head lolling and camptocormia (forward bending of the neck and trunk) in older individuals. Yet another pattern is of early diaphragmatic weakness; such cases come to attention because of respiratory failure. A symmetrical proximal limb or shoulder-girdle amyotrophy with onset at an early age is also known and

Primary iliac muscle abscess due to Staphylococcus aureus.

A 55-year-old man presented with a 3-day history of lower back pain and right thigh pain. A diagnosis of discogenic pain had been made at two other hospitals. He had been admitted to a medical center for acute hepatitis 5 months prior to this admission. Large doses of parenteral hydrocortisone were used for 13 days to treat acute hepatitis. At the present admission, he was unable to stand and refused to move his right leg. There was mild tenderness in the right lower abdomen on deep palpation. Passive flexion and rotation of the right hip produced mild pain, while passive extension of the right hip produced severe pain and resistance. The Patrick test was positive and the psoas sign was present on the right side. The erythrocyte sedimentation rate (ESR) was 66/hr. The C-reactive protein (CRP) level was 0.161 g/L. Abdominal sonography showed a lobulated mass in the right iliac fossa. Magnetic resonance imaging showed severe swelling of the right iliac muscle with a central heterogeneous mass. Debridement, drainage of the abscess, and application of a septopal chain were performed via an anterior retroperitoneal approach, and parenteral cephazolin and gentamicin were administered. A culture of the abscess grew Staphylococcus aureus. The ESR and CRP concentrations decreased to within the normal ranges 3 weeks later. Awareness of this disease entity, careful physical examination, and appropriate imaging studies such as ultrasonography and magnetic resonance imaging are key to making a correct diagnosis.

[Value of the so-called "unravelling" Doppler test at the level of the carotid axes during an examination of a lower-limb arteriopathy].

The use of routine, detailed, complete cervical Doppler examinations in patients with lower limb arteriopathy is criticized, based on results in a prospective study of 676 cases. A more compact, rapidly performed examination known as unravelling Doppler is defined for subclavian and carotid axis arterial investigation. Results are assessed based on a prospective study of 330 patients. A selective practical attitude is proposed by definition of two groups of patients: those with the highest risk of presenting cervical arterial lesions (a routine, complete Doppler test is conducted), and those, the most numerous, for whom the unravelling Doppler enables selection of cases most liable to benefit from a more complete examination of axes supplying the brain.

[A case of flexion myelopathy presenting juvenile segmental muscular atrophy of upper extremities--a successful treatment by cervical spine immobilization].

A 24-year-old man was well until 1982, when he noticed weakness and atrophy of right arm especially those of biceps muscles. These symptoms did not progress until 1988. In 1988 weakness of left elbow flexion appeared and he was admitted to our hospital. On examination, he had weakness and atrophy of both upper extremities. Fasciculation was noticed in the proximal part of right arm. When his head was bent forward, dysesthesia appeared around radial side of his arm. There were no long tract sign or objective sensory loss. EMG showed neurogenic change over both his upper extremities. In plain cervical roentgenogram there existed abnormal kyphosis of cervical vertebrae. In MRI imaged with his neck flexed, cervical cord was compressed by the posterior surface of 4th cervical vertebral body, which corresponded to the top of cervical kyphosis. Based on these signs and examinations, a diagnosis of flexion myelopathy was made. Spinal immobilization by wiring and bone graft implantation ranging from C2 to C6 was performed to limit the range of anterior nuchal flexion. After this operation, improvement of muscle strength along with the disappearance of dysesthesia on neck flexion was observed. Clinically, this case closely resembles to juvenile type of distal and segmental muscular atrophy of upper extremities (Hirayama type). From our study, pathomechanism of this case seems to be a flexion myelopathy at C4 level. This view is supported also by the good recovery after the operation. This case suggests that nonprogressive atrophy of upper extremities due to flexion myelopathy can be successfully treated by cervical spine immobilization.

InternalMed_Harrison

Fever, constant pain uninfluenced by position, sphincter abnormali-115 ties, or signs of spinal cord disease suggest an etiology other than lumbar disk disease. Absence of ankle reflexes can be a normal finding in persons older than age 60 years or a sign of bilateral S1 radiculopathy. An absent deep tendon reflex or focal sensory loss may indicate injury to a nerve root, but other sites of injury along the nerve must also be considered. For example, an absent knee reflex may be due to a femoral neuropathy or an L4 nerve root injury. A loss of sensation over the foot and lateral lower calf may result from a peroneal or lateral sciatic neuropathy or an L5 nerve root injury. Focal muscle atrophy may reflect injury to the anterior horn cells of the spinal cord, a nerve root, peripheral nerve, or disuse.