bluuebunny/crossref_metadata_2025_split huggingface dataset:
Transfusion‐associated AIDS
Authors: Perkins Herbert A. 
Published: February 2007

AbstractAcquired Immunodeficiency Disease (AIDS) may be transmitted by transfusion of blood components, although the risk remains extremely small. This report reviews the evidence for transmission by transfusion, the risk in several different situations, and the steps blood banks have taken to prevent transfusion of blood from donors at risk for AIDS. A test for the antibody to the virus responsible for AIDS (HTLV‐III) will shortly be in routine use in blood banks, but the frequency with which the results will be falsely negative or falsely positive in a blood donor population remains to be established.
A case of factor V inhibitor
Authors: Chong Lai‐Lu , Wong Yuk‐Cheung 
Published: February 2007

AbstractA 57‐year‐old married Chinese male without a family history of bleeding disorder was presented with severe hemorrhagic tendency and was subsequently found to be suffering from an acquired inhibitor against coagulation factor V. The prolonged prothrombin time and activated partial thromboplastin time could not be corrected by the addition of normal plasma. Subnormal value of factor V level was noted accompanied with an abnormal platelet factor III availability test. With specific antisera and staphylococcal protein A, the inhibitor was characterized as an IgG(lambda) antibody. The hemorrhagic tendency and abnormal laboratory data were corrected after treating the patient with platelet concentrate transfusion and cyclophosphamide.
Therapy of chronic relapsing thrombotic thrombocytopenic purpura with prednisone and azathioprine
Authors: Moake Joel L. , Rudy Christine K. , Troll Joseph H. , Schafer Andrew I. , Weinstein Mark J. , Colannino Noreen M. , Hong Suchen L. 
Published: February 2007

AbstractAs a 51‐year‐old woman recovered from an initial acute episode of thrombotic thrombocytopenic purpura (TTP), her plasma was found to contain unusually large von Willebrand factor (vWF) multimers. Clinical, hematological, and vWF studies of her siblings and children were normal. The unusually large vWF forms were presumably derived from endothelial cells, persisted in her plasma after recovery, and were associated with recurrent episodes of TTP during the subsequent 6 months. After the last episode of relapse they disappeared from her plasma following 3 ½ weeks of therapy with prednisone and did not return during 17 months of treatment with prednisone and/or azathioprine. She is now receiving no drugs, has normal plasma vWF forms, and has not had any more episodes of TTP. We conclude that our patient had an acquired defect in the conversion of unusually large vWF multimers derived from endothelial cells to the somewhat smaller vWF forms usually present in circulation. The defect may have been immune‐mediated, because it was eliminated during therapy with immunosuppressive drugs.
Clonal development from a progenitor with restricted differentiative expression in acute lymphoblastic leukemia
Authors: Ferraris Anna Maria , Canepa Letizia , Massimo Luisa , Dini Giorgio , Broccia Giorgio , Meloni Tullio , Forteleoni Gavino , Melani Cecilia , Gaetani Gian Franco 
Published: February 2007

AbstractThree patients with acute lymphoblastic leukemia (ALL) and heterozygous for the Mediterranean variant of glucose‐6‐phosphate dehydrogenase (G6PD) have been investigated with the 2‐deoxyglucose‐6‐phosphate (2dG6P) method to determine the number and type of progenitor cells in which the disease arose. A monoclonal origin was established for the lymphoblasts, while the other hemopoietic cell lines were not involved in the leukemic process.
Release of granulocyte‐macrophage colony‐inhibiting activity by normal human postthymic precursor cells
Authors: López‐Karpovitch X. , Padrós‐Semorile M. R. , Rojas R. , Martínez‐Sánchez L. 
Published: February 2007

AbstractSeven normal human peripheral blood cell fractions (buffy coat, mononuclear cells, non‐T, T, Fc‐IgM receptor‐depleted T‐lymphocyte, Fc‐IgG receptor‐depleted T‐lymphocyte, and autologous rosette‐forming T‐cell‐depleted T‐lymphocyte subpopulations) treated with phytohemagglutinin (PHA) were examined for the production of granulocyte‐macrophage colony‐stimulating activity (CSA). It was found that medium conditioned by a T‐lymphocyte subpopulation depleted of autologous rosette‐forming T‐cells (Tar cells, a postthymic precursor subpopulation that inhibits Ig synthesis) stimulated colony‐forming units of granulocyte and macrophages (CFU‐GM) to a greater extent than did the other conditioned media (CM) analyzed. Based on this finding, CM from an enriched Tar subpopulation was prepared and thus showed that PHA‐treated Tar cells release a factor capable of inhibiting CFU‐GM growth. The inhibitory activity of this factor persisted after heat inactivation, suggesting that cause of the colony‐inhibiting activity (CIA) is other than interferon. Further studies revealed that Tar‐derived inhibitory factor acts either directly upon CFU‐GM or via monocytes/macrophages (Mϕ/Ma), enhancing CIA, and not the level of CSA production by Mϕ/Ma. The overall data are interpreted as demonstrating the presence of CIA in a specific T‐lymphocyte subpopulation that may represent a new relationship between lymphocytic and myelocytic systems in the human.
A subpopulation of suppressor cells in Richter's syndrome with both monocytic and T‐lymphocytic characteristics
Authors: Lahat N. , Aghai E. , Quitt M. , Nir E. , Froom P. 
Published: February 2007

AbstractWe evaluated T‐lymphocyte functions in the peripheral blood of a patient with B‐cell chronic lymphocytic leukemia after transformation to large cell lymphoma (Richter's syndrome). A subpopulation of E‐rosette adherent cells were found with T‐lymphocytic surface markers (OKT3+/8+/4+), monocytic characteristics (latex ingestion, nonspecific esterase staining), and suppressor activity. In contrast to the patient's nonadherent T‐cells, this subpopulation suppressed PHA proliferation of autologous lymphocytes, pokeweed mitogen (PWM)‐induced proliferation of normal non‐T cells, and a mixed lymphocyte reaction. Further studies are warranted in patients with Richter's syndrome, in order to determine the frequency and significance of our findings.
An increased calcium accumulation in ATP‐depleted red cells of the patients with paroxysmal nocturnal hemoglobinuria
Authors: Shimoda Masaoo , Yawata Yoshihito 
Published: February 2007

AbstractA marked increase in calcium (Ca) uptake (85.5 ± 39.0 nmoles/ml RBC/2 hr) was observed in ATP‐depleted red cells of patients with paroxysmal nocturnal hemoglo‐binuria (PNH) as compared to ATP‐depleted normal red cells (8.0 ± 2.0). The extent of increased Ca uptake in the PNH red cells was related to the extent of increased sensitivity to complement, as judged by the sugar water test (p < 0.001) and the acidified serum test (p < 0.001), and inversely correlated to the decreased activity of red cell acetylcholinesterase (p < 0.01). The results may indicate that increased calcium uptake in PNH red cells is a possible intrinsic defect of the red cell membranes in the disorder.
Transferrin sialic acid contents of patients with hereditary hemochromatosis
Authors: Tsan Min‐Fu , Scheffel Ursula 
Published: February 2007

AbstractRecent evidence suggests that asialotransferrin may enhance hepatic uptake of trivalent metal ions through the asialoglycoprotein‐receptor pathway. In hereditary hemochromatosis, there is preferential uptake of iron by hepatocytes. We purified transferrin from two patients with this disease. Their transferrins had normal electrophoretic patterns and contained normal amounts of sialic acid. We conclude that the preferential uptake of iron by hepatocytes in hereditary hemochromatosis is not due to the presence of asialotransferrin in their plasma.
The incidence of thrombotic and hemorrhagic disorders in association with extreme thrombocytosis: An analysis of 129 cases
Authors: Buss David H. , Stuart John J. , Lipscomb Gary E. 
Published: February 2007

AbstractWe have presented a retrospective study of 129 patients with platelet counts of 1,000 × 109/L or more—72 with myeloproliferative disorders (MPD), and 57 with reactive thrombocytosis (RT)—in an effort to determine the incidence of thrombohemorrhagic complications. Thrombotic disorders occurred with approximately equal frequency in the two groups, being found in three patients (4%) with MPD and three (5%) with RT when the platelet count was over 1,000 × 109/L and in 11% of the patients in each group when the platelet count was less than 1,000 × 109/L. In contrast, bleeding manifestations were more common in MPD, where 36% of patients were affected on one or more occasions versus only 4% of those with RT. However, bleeding was generally not severe, with only six patients requiring transfusions, and three of these patients had been receiving aspirin or corticosteroids when they developed severe gastrointestinal bleeding.
Adult Niemann‐Pick disease masquerading as sea blue histiocyte syndrome: Report of a case confirmed by lipid analysis and enzyme assays
Authors: Landas Steve , Foucar Kathy , Sando Gloria N. , Ellefson Ralph , Hamilton Henry E. 
Published: February 2007

AbstractWe present the clinical, pathologic, and metabolic findings of an adult woman with debilitating coronary artery disease and hepatosplenomegaly who was discovered to have multiorgan infiltration by sea blue histiocytes. A diagnosis of sea blue histiocyte (SBH) syndrome was made and no further workup performed. The patient suffered from progressive heart failure and sepsis following coronary artery bypass surgery and died 9 months after presentation. Tissues examined at autopsy showed pronounced infiltrates of both granular sea blue histiocytes and foamy, vacuolated histiocytes, which were morphologically compatible with Niemann‐Pick cells. Ultra‐structural examination of these cells revealed lamellar myelin‐like figures as described in Niemann‐Pick (N‐P) disease. Fibroblast enzyme assay studies and liver lipid analyses performed after the patient's death revealed pronounced sphingomyelinase deficiency and a lipid profile diagnostic of N‐P disease, type B. This case adds further support to the claim that some cases of apparent SBH syndrome actually represent a type of N‐P disease.
Post‐transfusion purpura associated with alloimmunization against the platelet‐specific antigen, baka
Authors: Keimowitz Rudolph M. , Collins Janice , Davis Kent , Aster Richard H. 
Published: February 2007

AbstractPost‐transfusion purpura (PTP) with severe thrombocytopenia occurred eight days after transfusion in a 28‐year‐old woman and responded to treatment with prednisone and plasma exchange. In contrast to nearly all previously studied cases of PTP, the patient's platelets were PIAI‐positive and anti‐PIAI antibody could not be detected in serum obtained during the thrombocytopenic episode. Her serum was found to contain an antibody specific for a recently described platelet‐specific alloantigen, Baka, in addition to multiple HLA‐specific antibodies. The patient's platelets, typed following recovery, were Baka‐negative. These findings indicate that post‐transfusion purpura can occur in association with alloimmunization to platelet‐specific antigens other than PIAI.In performing the serologic studies, a close relationship and possible identity between Baka and another recently reported platelet antigen, Leka, was observed. A method for analyzing mixtures of cytotoxic platelet‐reactive antibodies without separating the individual antibodies is described.
The distribution of lysozyme, alpha‐1‐antitrypsin, and alpha‐1‐antichymotrypsin in normal hematopoietic cells and in myeloid leukemias: An lmmunoperoxidase study on cytocentrifuge preparations, smears, and paraffin sections
Authors: Krugliak Lena , Meyer Paul R. , Taylor Clive R. 
Published: February 2007

AbstractBlood and bone marrow samples from 20 individuals with reactive conditions and 26 cases of acute and chronic myeloid leukemias were tested for the presence of lysozyme, alpha‐1‐antitrypsin (α‐1‐AT), and alpha‐1‐antichymotrypsin (α‐1‐ACT). We compared the reactivity of samples in smears, cytocentrifuge preparations, and paraffin sections.Lysozyme, alpha‐1‐antitrypsin, and alpha‐1‐antichymotrypsin were found only in polymorphonuclear leukocytes and monocytes and their precursors. Lymphocytes, Erosetting cells, Con A‐activated lymphocytes, natural killer (NK) cells, red blood cells, erythroblasts, and megakaryocytes were consistently negative.Leukemic myeloblasts showed definite reactivity for both alpha‐1‐antitrypsin and α‐1‐ACT, but not for lysozyme. By contrast, lysozyme was present in poorly differentiated leukemic monoblasts, while alpha‐1‐antitrypsin and alpha‐1‐antichymotrypsin showed only weak reactivity. More mature myeloid and moncytic cells showed positive staining for all three antigens tested with differences in staining distribution and intensity.In four cases of chronic myeloid leukemia (CML), circulating mature polymorphonuclear leukocytes were deficient in both lysozymne and alpha‐1‐antitrypsin.The use of lysozyme, alpha‐1‐antitrypsin, and alpha‐1‐antichymotrypsin identifies normal and leukemic cells of the myeloid‐monocytic series at all stages of maturation and is applicable to a variety of sample preparations.
Successful renal transplantation for epstein syndrome
Authors: Alving Barbara M. , Tarassoff Peter G. , Moore Jack , Leissenger Cindy A. , Fernandez‐Bueno C. 
Published: February 2007

AbstractSuccessful cadaveric renal transplantation was accomplished in a patient with Epstein syndrome, a triad of macrothrombocytopenia, partial high‐frequency hearing loss, and nephritis, which often progresses to complete renal failure. The success of the transplant demonstrates that the macrothrombocytopenia which occurs in this syndrome is not a contraindication to aggressive management of end‐stage renal disease.
Hemoglobin knossos: A clinical, laboratory, and epidemiological study
Authors: Fessas Phaedon , Loukopoulos Dimitris , Kokkinou Stavroula , Papasotiriou Yannis , Karaklis Aris 
Published: February 2007

AbstractHb Knossos is a β‐chain variant (β 27 Ser ± Ala) that is unrecognizable by conventional separation methods but detectable by globin electrophoresis on urea‐Triton X‐acrylamide gels or by IEF. Hb Knossos is characterized by reduced synthesis and by interaction with β‐thalassemia, in which the double heterozygotes display typical features of thalassemia intermedia. The present paper summarizes the salient genetic, clinical, and biochemical characteristics of five such cases hitherto identified in three families along with the same features on 12 heterozygous Hb Knossos carriers. Hb Knossos displays a slightly decreased oxygen affinity; this factor may compensate in part for the severe anemia of the double heterozygotes. Hb Knossos is relatively rare in our population, since a prospective survey on 610 individuals has failed to disclose any heterozygotes. However, the mutation appears to have spread over the Mediterranean countries and may be more common elsewhere.
T‐cell chronic lymphocytic leukemia with a helper/inducer membrane phenotype: A distinct clinicopathologic subtype with a poor prognosis
Authors: Witzig Thomas E. , Phyliky Robert L. , Li Chin‐Yang , Homburger Henry A. , Dewald Gordon W. , Handwerger Barry S. 
Published: February 2007

AbstractT‐cell chronic lymphocytic leukemia (T‐CLL) accounts for about 2% of the various types of CLL and can be subtyped into helper/inducer (h/i) and cytotoxic/suppressor (c/s) cell membrane phenotypes. Seven patients with CLL were shown to have T‐CLL with a h/i cell membrane phenotype; four with monoclonal antibody reagents and three by demonstration of the E‐rosette receptor and focal acid alpha naphthyl acetate esterase activity. The clinical courses, treatment responses, and laboratory findings of these seven patients were reviewed to determine the prognosis and unique clinicopathologic features of this subtype. Two patients presented with skin rashes, and five were diagnosed during evaluation for other medical problems. Initially, four patients had splenomegaly and two had lymphadenopathy, but none of the patients had hepatomegaly. Morphologic examination revealed uniform, small lymphocytes in three patients, and the lymphocytes had nuclear indentations in four patients. Sera from the three patients tested were negative for antibody to the human T‐cell leukemia/lymphoma virus I. Peripheral blood mononuclear cells from one patient showed normal interleukin‐2 production and lacked antibody‐dependent cell‐mediated cellular cytotoxicity and natural killer activity. Cytogenetic analysis was done on one patient, revealing an abnormal clone with several chromosomal abnormalities, including an X; 14 translocation with a break point at 14q11. All patients required chemotherapy, and all died a median of 21 months from the time of diagnosis. The findings in these patients, in addition to those in 31 patients described in the literature, indicate that h/i T‐CLL is associated with a poor prognosis and has distinct clinical and pathologic features that separate it from c/s T‐CLL, adult T‐cell leukemia/lymphoma, the cutaneous T‐cell lymphomas, and B‐CLL.
von willebrand disease type IIC with different abnormalities of von willebrand factor in the same sibship
Authors: Batlle J. , Fernandez M. F. Lopez , Lasierra J. , Villamor A. Fernandez , Berges C. Lopez , Borrasca A. Lopez , Ruggeri Z. M. , Zimmerman T. S. 
Published: February 2007

AbstractA family with von Willebrand disease has been identified in which different members of the same sibship exhibit different abnormalities of von Willebrand factor (vWF). The two most severely affected sibs (bleeding time over 20 min) had abnormalities of vWF similar to those seen in type IIC. The smallest detectable multimer was increased and the triplet structure of individual multimers was replaced with a single band. The largest multimers could not be detected and there were relatively more small multimers than intermediate sized forms. vWF antigen (vWF:Ag) was decreased to 12.5‐17% by electroimmunoassay (EIA) and to 3.2‐5.5% by immunoradiometric assay (IRMA). In the less severely affected sibling (bleeding time 12.5 min) there was a similar relative increase in the smallest detectable multimer. However, the larger multimers were present and the relative concentration of large to small multimers was similar to normal. The triplet structure was altered in that the relative proportion of satellite bands to the central predominant band was decreased. vWF:Ag concentrations were moderately decreased (40‐80% by EIA and 25‐35% by IRMA). The father and grandfather showed a vWF multimeric pattern similar to the less severely affected sibling but there was no decrease in vWF:Ag concentration and their bleeding times were normal. These observations suggest that the interplay of several genetic factors is responsible for the expression of von Willebrand disease in this family.
Hereditary antithrombin III deficiency: Case report and review of recent therapeutic advances
Authors: Miller Nancy , Hultin Mae B. , Gounder Mohanambal , Zarrabi M. Hosein 
Published: February 2007

AbstractWe report on a newly diagnosed family with hereditary antithrombin III deficiency, with thromboembolic complications in the propositus. Both the propositus and his asymptomatic sister had decreased plasma levels of antithrombin III antigen and activity (28‐52% of normal with good agreement between functional and immunologic assays). The propositus developed deep venous thrombosis, followed by massive pulmonary emboli despite heparin therapy and was treated with streptokinase and heparin with excellent results. Shortly thereafter, small bowel obstruction required surgical intervention, and antithrombin III concentrate, recently available in the United States as an investigational new drug (I. N. D.), was administered with no postoperative thrombotic complications. He was subsequently asymptomatic while on warfarin prophylaxis but twice developed venous thrombosis when he failed to take warfarin. The addition of danazol therapy led to a sustained rise in the antithrombin III level. Each of these therapeutic approaches is discussed and the literature reviewed with emphasis on the newer agents‐streptokinase, antithrombin III concentrate, and danazol.
Hematologic malignancy in sickle cell disease: Report of four cases and review of the literature
Authors: Stricker Raphael B. , Linker Charles A. , Crowley Timothy J. , Embury Stephen H. 
Published: February 2007

AbstractHematologic malignancy has rarely been reported in adults with sickle cell disease. We describe four sickle cell patients (two with hemoglobin SC, two with hemoglobin SS) who developed hematologic malignancy (acute myeloblastic leukemia, multiple myeloma, malignant histiocytosis, and Hodgkin's disease). Three of the cases represent the first adult association between SC or SS hemoglobinopathy and the particular malignancy involved. Sickle hemoglobin does not appear to exert a protective effect against childhood hematologic malignancies, suggesting that better survival in sickle cell disease may be accompanied by an increased incidence of hematologic neoplasms in adulthood. Karyotypic analysis revealed alterations of chromosome 5 in two sickle cell patients with leukemia, raising the possibility of a chromosomal link between the two diseases. Further epidemiologic and cytogenetic studies are needed to define the relationship between hematologic malignancy and sickle cell disease.
Post‐transfusion purpura: A report of five patients and a review of the pathogenesis and management
Authors: Vogelsang Georgia , Kickler Thomas S. , Bell William R. 
Published: February 2007

AbstractWe report five cases of post‐transfusion purpura. Despite having profound thrombocytopenia, their management consisted only of steriod administration if serious bleeding was present. In one patient without bleeding, no specific therapy was given. This patient recovered uneventfully. Two patients died. One of these died from complicating illnesses rather than bleeding. The other suffered brain death, the result of a ruptured cerebral aneurysm. Four patients were PLA1 negative. The fifth patient had an antibody to an unidentified platelet‐specific antigen.
Thrombotic thrombocytopenic purpura subsequent to acute myelogenous leukemia chemotherapy
Authors: Byrnes John J. , Baquerizo Hernan , Gonzalez Michael , Hensely George T. 
Published: February 2007

AbstractA woman in complete remission from acute myeloblastic leukemia developed thrombotic thrombocytopenic purpura (TTP) subsequent to the third intensive consolidation cycle of cytosine arabinoside and daunorubicin chemotherapy. The constellation of clinical manifestations indicative of TTP were recognized only in retrospect, as they were initially attributed to more usual complications of bone marrow‐ablative chemotherapy. The manifestations, probably fueled by numerous red cell and platelet transfusions, increased at the time of recovery of hematopoiesis. At postmortem examination, characteristic microvascular lesions were found in most organs. Similar thrombotic microangiopathy has been described with mitomycin‐based chemotherapy regimens and with the combination of cisplatin, vinblastine, and bleomycin. Successful management of this serious complication of chemotherapy requires increased awareness and earlier recognition.
Common variable hypogammaglobulinemia in pregnancy: Treatment with high‐dose immunoglobulin infusions
Authors: Madsen Douglas L. , Catanzarite Valerian A. , Varela‐Gittings Frances 
Published: February 2007

AbstractPregnancy outcomes when the mother has common variable hypogammaglobulinemia are poor unless immunoglobulin levels are returned to normal or near‐normal range. Intravenous immunoglobulin infusions are the favored mode of therapy. Here, we report the fifth patient so treated. Weekly doses of 200 mg/kg during the third trimester were required. A healthy term baby was delivered and the postpartum course was uncomplicated.