bluuebunny/crossref_metadata_2025_split huggingface dataset:
Autoimmune hemolytic anemia and the kell blood groups
Authors: Marsh W. Laurence , Øyen Ragnhild , Alicea Edith , Linter Meredith , Horton Susan 
Published: February 2007

AbstractApproximately one in 250 people with autoimmunity involving their red cells have IgG autoantibodies with specificity in the Kell blood groups. Red cells of these individuals have an acquired temporary weakening of their Kell antigens. Some of the patients also have allo‐anti‐K in their serum. This report presents a case in which an IgG autoantibody may define a new high‐incidence red cell antigen related to the Kell blood groups. The patient's Kell blood group antigens are depressed, and his serum contains allo‐anti‐K. It is postulated that reduced red cell Kell antigenicity is caused by enzymatic degradation, possibly of bacterial origin, and that the acquired loss of Kell antigens, the Kell‐specific autoimmune state, and the serum allo‐anti‐K, are all related aspects of one phenomenon.
Fetal to adult hemoglobin switch in cultures of early erythroid precursors from human fetuses and neonates
Authors: Beuzard Yves , Vainchenker William , Testa Ugo , Dubart Anne , Monplaisir Nicole , Breton‐Gorius Janine , Rosa Jean , Bouguet Jacqueline , Cacheleux Janine , Rouyer‐Fessard Philippe 
Published: February 2007

AbstractErythroid burst colonies derived from the cord blood of nine neonates and from the blood and liver of three fetuses aborted after 20 weeks of gestation were grown in plasma clot culture. Their quantitative study revealed a higher proportion of burst‐forming units (BFU‐Es) in cord blood than in cord blood of normal adults. In addition, colony‐forming units (CFU‐Es) were present in cord blood but absent from adult blood. Study of haemoglobin synthesis in 14‐day cultures of cord blood BFU‐Es showed a significantly higher degree of Hb A synthesis than was found in reticulocytes from fresh cord blood; this proportion was, however, similar to that expected in vivo about three weeks after birth. These data suggest that the hemoglobin switch is already programmed in most of the early erythroid precursors present in cord blood of full‐term neonates and indicate that the differentiation time is probably of the same order of magnitude in vivo and in vitro. The proportion of Hb A and F synthesis in erythroid bursts was not influenced by the concentration of erythropoietin in the range studied – ie, from 0.5 to 12 international units. Low but identical proportions of Hb A synthesis were found both in erythroid cells from liver after two hours of incubation with [3H]‐leucine, and in 14‐day liver bursts from fetuses aborted at 20 weeks of gestation.
The diagnosis of pulmonary thromboembolism in sickle cell disease
Authors: Walker Brian K. , Ballas Samir K. , Burka Edward R. 
Published: February 2007

AbstractThe symptoms of thromboembolism in sickle cell anemia patients with acute chest syndromes are difficult to differentiate from the similar symptoms of painful thoracic crises and infectious pulmonary episodes. Furthermore, the chronic pulmonary abnormalities in sickle cell disease frequently contribute to the confusing results of noninvasive diagnostic procedures usually employed in evaluating pulmonary thromboembolism. In this study the chronic pulmonary status of asymptomatic sickle cell patients was defined, and this information was used in the evaluation of patients with acute chest syndromes suggestive of pulmonary thromembolism. Sixteen asymptomatic sickle cell patients were prospectively studied by chest roentgenography, spirometry, arterial gas analyses, and radioisotopic lung scans. There was an appreciable degree of preexisting chronic restrictive lung disease with mild to moderate arterial hypoxemia and abnormal lung scans in more than half of the patients. These prospective baseline data were incorporated into the diagnostic evaluation of four of these patients who later developed an acute chest syndrome suggestive of pulmonary thromboembolism. Determination of the cause of the chest pain was greatly facilitated by the existence of the baseline pulmonary data. In another patient persistence of abnormal studies following a presumed thromboembolic episode aided diagnostic evaluation when another episode of chest pain occurred. The findings indicate that comprehensive pulmonary studies in sickle cell patients while in an asymptomatic state will provide baseline data which aid the evaluation of possible pulmonary thromboembolism in acute chest syndromes without resorting to high‐risk invasive studies.
Oxygen dissociation curves in children with anemia and malignant disease
Authors: Festa Robert S. , Asakura Toshio 
Published: February 2007

AbstractTwo automatic apparatuses utilizing a dual wavelength spectrophotometer were used to perform oxygen dissociation curves on microsamples of blood. The method provides a complete print‐out of an oxygen dissociation curve in 15–20 min and the P50 values obtained in normal individuals agree closely with those obtained by classical methods. These apparatuses were used to measure oxygen affinity, ie P50, in anemic children with malignant disease prior to treatment and in children undergoing therapy. Red cell 2,3‐DPG levels were also measured. In patients with anemia at the time of diagnosis and prior to therapy, the P50 values and 2,3‐DPG levels were elevated as is usual in other types of anemia. However, when oxygen affinity and 2,3‐DPG levels were measured in anemic patients receiving treatment, three types of response to anemia were noted: 1) increased P50 and 2,3‐DPG; 2) normal or low P50 and 2,3‐DPG, and; 3) normal or low P50 with increased 2,3‐DPG. Patients who adapted poorly to anemia during treatment had usually received prior intensive chemotherapy and transfusion therapy, and their pattern of red cell glycolytic intermediates was consistent with a red cell population with an increased cell age. The failure of some patients to respond to anemia with a decrease in oxygen affinity has implications in regards to the hemoglobin level at which they should be transfused.
Mechanisms of thrombocytopenia in varicella
Authors: Feusner James H. , Slichter Sherrill J. , Harker Laurence A. 
Published: February 2007

Abstract51Cr‐labeled platelet and 125I‐labeled fibrinogen kinetic studies in four thrombocytopenic children with varicella infection revealed marked platelet destruction (platelet survival times 0.1, 0.4, 1.0, and 2.4 days) and relatively normal fibrinogen disappearance. The platelet count was directly related to the platelet survival time. Marrow megakaryocyte mass was increased twofold to fourfold. IgG or IgM antiplatelet antibody was present on autologous platelets in the three patients tested. These data suggest that thrombocytopenia in some patients with varicella is the consequence of immune mediated platelet destruction.
(Ca2++Mg2+)‐ATPase activity of sickle cell membranes: Decreased activation by red blood cell cytoplasmic activator
Authors: Gopinath Rama M. , Vincenzi Frank F. 
Published: February 2007

AbstractHuman red blood cells (RBCs) contain a cytoplasmic protein that activates membrane‐bound (Ca2+ + Mg2+)‐ATPase and the transport of Ca2+. The (Ca2+ + Mg2+)‐ATPase of sickle cells showed a less than normal response to this activator. This was true whether the activator was obtained from normal or sickle cells. Activator present in sickle cell hemolysates fully activated the (Ca2+ + Mg2+)‐ATPase of normal RBC membranes. These results demonstrate that membranes of sickle cells are defective in their response to the activator. Neither the apparent affinity for calcium nor the apparent affinity for activator was different comparing the (Ca2+ + Mg2+)‐ATPase of sickle and normal membranes. Young, mature, and irreversibly sickled cells were separated by density gradient centrifugation, and membranes were prepared from each of these cell populations. No significant differences in ATPase activities were found based on cell age (density). The (Ca2+ + Mg2+)‐ATPase of all populations of sickle cells showed a decreased response to the activator. Thus, it appears unlikely that the decreased response of the (Ca2+ + Mg2+)‐ATPase of sickle cells is due to membrane damage caused by repeated sickling during the life‐span of the cell. Reduced activation of (Ca2+ + Mg2+)‐ATPase by the cytoplasmic activator may account for calcium accumulation in sickle cells.
Abnormal lymphocyte deoxyuridine suppression test: A reliable indicator of decreased lymphocyte folate levels
Authors: Colman Neville , Herbert Victor 
Published: February 2007

AbstractThe deoxyuridine (dU) suppression test measures the availability of folate and, indirectly, vitamin B12 in de novo thymidine synthesis and is of established value in defining biochemical megaloblastosis in bone marrow cells. Recent studies have suggested that the application of this test to DNA synthesis in transformed lymphocytes may be valuable in defining nutrient deficiency that is subtle, masked, or present in some cell lines but not in others. This use of the test has not been fully accepted because lymphocyte folate and B12 levels had not been simultaneously measured prior to the current study. In this study, vitamin availability for the dU suppression test was closely correlated with folate levels in radioassay and less well correlated with vitamin B12 levels in the cell. The folate levels varied widely when dU suppression was efficient but were uniformly low in subjects with ineffective dU suppression. These studies validate the use of the lymphocyte dU suppression test as a means of detecting folate deficiency in this cell line.
Quantitative assessment of bactericidal ability of leukemic cells in childhood acute leukemia
Authors: Komiyama Atsushi , Saitoh Hiroharu , Kawai Hiroshi , Tsukada Masashige , Akabane Taro 
Published: February 2007

AbstractBactericidal ability of leukemic cells was evaluated in four children with acute myeloblastic leukemia (AML) and in two with acute monocytic leukemia (AMoL) by means of quantitative assay of intracellular viable bacteria and by electron microscopy. The electron microscopy confirmed phagocytosis of Staphylococcus aureus by the leukemic cells. The leukemic cells killed a significantly smaller proportion of phagocytized bacteria than normal neutrophils. In some patients the leukemic cells virtually lacked bactericidal ability, despite presence of phagocytic ability. The defect of the cells in killing bacteria was more prominent in AML than in AMoL.The clinical importance of the defective bactericidal ability of leukemic cells is discussed.
Mechanism of arsenic‐induced inhibition of erythropoiesis in mice
Authors: Morse Bernard S. , Conlan Maureen , Giuliani Dennis G. , Nussbaum Murray 
Published: February 2007

AbstractAnemia accompanies arsenic intoxication in man. The present studies were undertaken to clarify further the effects of arsenic on erythropoiesis. A dose‐related inhibition of red cell 59Fe incorporation and reticulocyte response was observed in normal mice treated with a single injection of arsenic. Arsenite was approximately two times as inhibitory as arsenate. The effects of arsenic on erythropoietin‐induced erythroid differentiation revealed a significant inhibitory effect on young, proliferating marrow nucleated erythroid precursor cells. More mature, nonproliferating nucleated erythroid cells were resistant to the toxic action of arsenic. A dose‐related inhibitory effect of arsenic on DNA synthesis was observed in fetal liver nucleated erythroid cells incubated with 3H thymidine. Ineffective erythropoiesis as well as the megaloblastic morphology accompanying aberrant DNA synthesis – manifestations of arsenic toxicity in man – were not evident in the present studies.
Plasma beta‐thromboglobulin values in thrombocytopenic patients with acute leukemia
Authors: Kutti Jack , Zaroulis Charles G. , Kulpa Jolanta , Reich Lilian , Clarkson Bayard D. , Good Robert A. 
Published: February 2007

AbstractPlasma beta‐thromboglobulin (β‐TG) levels were measured in 14 healthy subjects and in 20 acute leukemia (AL) patients, newly diagnosed, with highly variable values for venous platelet counts. For healthy subjects the plasma β‐TG levels ranged 12–38 (mean 17) ng/ml. In this group of patients with AL, a highly significant positive correlation (P < 0.001) between the values for plasma β‐TG and venous platelet count was present.During a thrombocytopenic period, the plasma β‐TG concentration was measured in nine of the AL patients immediately before and 10 to 12 hours after platelet transfusion therapy. Fourteen platelet transfusions were administered when the patient's highest temperature of the day was <38.5° C, and 18 when the highest temperature of the day was <38.5°C. The mean pretransfusion and post‐transfusion β‐TG values for the 14 platelet transfusions were 7 ± 2 and 20 ± 5 ng/ml, respectively. The corresponding means for the 18 transfusions given to febrile patients were 5 ± 2 ng/ml and 11 ± 2 ng/ml, respectively. Of the pretransfusion values, 11/14 and 14/18 were below the control range.We conclude that the plasma β‐TG values are considerably lower in thrombocytopenic patients than in subjects with normal platelet counts. Further work should provide reference values for plasma β‐TG over a wide range of venous platelet counts.
Acute promyelocytic leukemia—clinical management of 15 patients
Authors: Daly Peter A. , Schiffer Charles A. , Wiernik Peter H. 
Published: February 2007

AbstractFifteen patients with acute promyelocytic leukemia (APL) were treated with anthracycline agents alone or combined with arabinosylcytosine (Ara C). Disseminated intravascular coagulation (DIC) was managed individually based on the presence of clinical as well as laboratory evidence of DIC. Ten patients received heparin. Eleven patients (73%) achieved complete remission (CR) but there were two early deaths from infection. The median duration of CR was 232 days (range, 41–780 days). Six patients were studied during ten relapses. In all cases, a morphologic picture more typical of acute myelocytic leukemia was seen during relapse but the DIC was similar in severity to that seen initially. Fever, without obvious infection and possibly due to the leukemic process, was a common presenting feature. Regenerating bone marrow aspirates in many patients who subsequently achieved CR without further treatment showed an inordinate number of promyelocytes, and this finding should not be immediately interpreted as disease resistant to therapy.
Relationship of SV40 T‐antigen expression in vitro to disorders of bone marrow function
Authors: Lubiniecki A. S. , Blattner W. A. , Dosik H. , McIntosh S. , Wertelecki W. 
Published: February 2007

AbstractSkin fibroblasts from patients with a variety of hematologic disorders were infected with SV40 virus in vitro in attempts to discover the reason for increased susceptibility of Fanconi anemia cells to this transforming virus. The proportion of skin fibroblasts expressing SV40 T‐antigen by immunofluorescent methods was elevated in 12 patients with Fanconi anemia and in seven of nine obligate heterozygous relatives. Elevated expression was also observed in three patients with other hematological disorders at high risk of acute nonlymphocytic leukemia, but was not apparent in seven sporadic aplastic anemia patients or four of their relatives. T‐antigen expression was elevated in about one‐half of patients with thrombocytopenia‐absent radius syndrome and related conditions, with familial aplastic anemia, and in their normal relatives. In the conditions under study, elevated T‐antigen expression seemed clearly correlated with predisposition to leukemia, which may be genetically determined, but it was not associated with cytogenetic or anemic manifestations.
Pseudo‐drumsticks in granulocytes of a male with a Yqh+ polymorphism
Authors: Moore Charleen M. , Weinger Ronald S. 
Published: February 2007

AbstractAn individual with normal male habitus, body proportions, and secondary sexual characteristics was admitted to the hospital with head trauma. A routine blood smear demonstrated that 36% of the granulocytes had “drumsticks.” Chromosomal analysis revealed a 46, XYqh+ karyotype. The extremely large Y chromosome was located by quinacrine fluorescence in the “drumstick” of the polymorphonuclear granulocytes. The presence of a large Y chromosome may thus produce pseudo‐drumsticks. Fluorescent staining can distinguish between true drumsticks bearing the inactive X of normal females and the pseudo‐drumsticks in a normal male produced by a large Y chromosome.
Acute unclassified leukemia. A clinicopathologic study with diagnostic implications of electron microscopy
Authors: Youness Ehsan , Trujillo Jose M. , Ahearn Michael J. , McCredie Kenneth B. , Cork Ann 
Published: February 2007

AbstractBy rigid cytological and cytochemical criteria, the diagnosis of acute and undifferentiated leukemia was established in 22 patients. According to defined criteria, the leukemic cells could not be classified by conventional light microscopic techniques employed in the study of hematopoietic tissue. Cytochemical studies including peroxidase, periodic acid schiff (PAS) and nonspecific esterase (alpha napthyl butyrate‐reacting esterase) stains were done on fresh bone marrow samples, and the percentage of positive leukemia cells for each of these stains was determined on 200 cells. In this series of leukemias, cytochemistry at the light microscope level did not contribute to further classification. Subsequent electron microscopic examination of bone marrow samples from these patients confirmed the immaturity and nuclear/cytoplasmic asynchrony of the leukemic cells. Several in vivo neoplastic markers, such as nuclear blebs, increased nuclear bodies, and cytoplasmic fibrillar bundles could be demonstrated in these cells. Fourteen cases from this series exhibited peroxidase‐positive developmental granule formation at the ultrastructural level and were reclassified as acute granulocytic leukemia (AGL). One case was reclassified as lymphoma (poor differentiated type), one case was diagnosed as acute monocytic leukemia (AMonoL), and six cases remained in the undifferentiated category (AUL). Clinical and laboratory features, response to treatment, and survival data were evaluated for these patients. This study demonstrated that electron microscopy is useful in the cytological diagnosis of human leukemia.
Factor VII deficiency
Authors: Ragni Margaret V. , Lewis Jessica H. , Spero Joel A. , Hasiba Ute 
Published: February 2007

AbstractThis report describes three patients with factor (F) VII deficiency: two adult siblings and an unrelated 5½‐month‐old child who succumbed after several central nervous system (CNS) hemorrhages. This event prompted a review of the literature concerning the incidence and characteristics of intracranial hemorrhage in congenital F VII deficiency.Of 138 patients reported to have F VII deficiency, only 75 were considered to have a true deficiency. There was a 1:1 sex distribution with a 19% incidence of consanguinity in the 63 families which these 75 patients represented.CNS hemorrhage occurred in 12 of the 75 proven factor‐deficient patients – an incidence of 16.0%. There was a 1.4:1 female predominance in this group, with a 44.4% incidence of consanguinity in their nine families. Except for one patient with hypertension, there was no history of preceding trauma or previous underlying CNS abnormality, though head trauma with a difficult vaginal delivery may have occurred in five infants. Diagnostic lumbar puncture or ventricular tap revealed bloody, xanthochromic cerebrospinal fluid in five.Five patients with F VII deficiency developed a CNS hemorrhage prior to 1 week of age, and none survived. Seven patients older than 1 week of age suffered such an event, and four of these survived. It is concluded that the greatest risk factor for development of CNS hemorrhage is trauma related to the birth process.
von Willebrand disease associated with familial thrombocytopenia and increased ristocetin‐induced platelet aggregation
Authors: Takahashi Hoyu , Nagayama Reizo , Hattori Akira , Ihzumi Toshio , Tsukada Tsuneyasu , Shibata Akira 
Published: February 2007

AbstractTwo cases of von Willebrand disease (vWD) associated with familial thrombocytopenia were reported. The proband (daughter) and her father showed thrombocytopenia with large platelets and decreased von Willebrand factor activity (VIIIR:WF). Factor VIII procoagulant activity (VIII:C) and factor VIII‐related antigen (VIIIR:AG) were normal, but both patients revealed an increased ristocetin‐induced platelet aggregation and a qualitative abnormality of the factor VIII protein, which was characterized by fast electrophoretic mobility of VIIIR:AG and an abnormal elution of factor VIII‐related activities on Sepharose 2B. DDAVP was hemostatically effective even in this thrombocytopenic patient undergoing a dental extraction.
An antigen expressed by cells of the myelo‐monocytic lineage
Authors: Foon Kenneth A. , Billing Ronald J. , Fitchen John H. , Belzer Michael , Drew S. Ian , Terasaki Paul I. 
Published: February 2007

AbstractWe describe an antigen(s) characterized by a heteroantiserum raised in rabbits against mature human granulocytes. This antigen was found on neutrophils, monocytes, platelets, acute and chronic myelocytic leukemia cells and on granulocyte‐macrophage progenitor cells grown in agar. It was not found on lymphocytes, eosinophils, erythrocytes, or erythroid progenitor cells. On the basis of tissue distribution and absorption studies, the antigen (tentatively designated the “myelo‐monocytic” antigen) is distinct from antigens previously identified on human neutrophils. Restriction of the “myelo‐monocytic” antigen to normal and malignant cells of the myelo‐monocytic series suggests that it may represent a normal differentiation antigen of the myelomonocytic lineage.
Karyotypic patterns and their clinical significance in polycythemia vera
Authors: Testa J. R. , Kanofsky J. R. , Rowley J. D. , Baron J. M. , Vardiman J. W. 
Published: February 2007

AbstractWe studied a series of 34 polycythemia vera (PV) patients to determine the incidence of chromosome abnormalities and their relationship to leukemic transformation. Metaphase chromosomes from bone marrow or unstimulated peripheral blood were examined with conventional stain and, in 20 patients, also with banding techniques. The overall incidence of clonal abnormalities in first samples was 44% (15 of 34 patients), with a higher incidence in patients who had received prior therapy. The most frequent abnormalities were long‐arm deletion of No. 5 (5q‐; four patients), gain of all or most of a No. 8 (five patients), gain of all or most of a No. 9 (four patients), and long‐arm deletion of No. 20 (three patients); these sometimes occurred in combination. Some of the PV patients who had cytogenetic abnormalities early in the disease have had long survivals without developing leukemia. For example, one patient has been 48, XX, +8, +9 in sequential samples obtained over the last 15 years. Five patients developed acute myeloblastic leukemia (AML); each had a complex chromosomal pattern. Two of these five patients were cytogenetically normal during the polycythemic phase; the change in karyotype occurred with the leukemic transformation. Three AML patients were studied only in the leukemic phase. Three AML patients had a 5q‐, and a fourth showed loss of a B‐group chromosome. Evolutionary changes in the karyotype during the disease course and the occurrence of a 5q‐appear to be associated with a terminal phase of PV.