bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical relevance of gestational thrombocytopenia of <100,000/μl
Authors: Anteby Eyal , Shalev Oded 
Published: February 2007

AbstractWe identified 22 women with thrombocytopenia of <100,000/μl found incidentally during pregnancy and prospectively monitored their platelet count and clinical outcome for a minimum of 6 months postpartum. During the study period, four women became pregnant twice, accounting for a total of 26 pregnancies. The lowest platelet count during pregnancy was 65,600/μl ± 19,400 (mean ± SD), and at delivery 84,500/μl ± 32,300 (P < 0.02). The thrombocytopenia was virtually asymptomatic in all patients during the pregnancy and delivery, whether vaginal or surgical. Neonatal platelet counts (n = 18) were normal (270,700/μl ± 69,900), and none of the newborns (n = 24) had a bleeding diathesis. Normalization of the platelet count (i.e., >150,000/μl) was documented in 18 patients within 1 month postpartum, in five within 3 months postpartum, and in two as late as 5 months after delivery. One woman did not recover from the thrombocytopenia and eventually developed other stigmata of an autoimmune disease. Long‐term follow‐up showed recurrence of thrombocytopenia in four patients: three in the context of a subsequent pregnancy and one who developed idiopathic thrombocytopenic purpura. Retrospective analysis of blood counts obtained from 12 previous pregnancies demonstrated thrombocytopenia of a similar degree to the index pregnancy. We conclude that gestational thrombocytopenia of <100,000/μl is clinically a benign phenomenon that can recur in subsequent pregnancies and is not accompanied by neonatal thrombocytopenia. In some cases, however, pregnancy‐associated thrombocytopenia may be a manifestation of an autoimmune disease with its attendant implications for the neonate. Since the differential diagnosis between the two conditions may be difficult to establish when first encountered during pregnancy, a conservative approach emphasizing careful surveillance and guarded reassurance is justified as long as the platelet counts are >50,000/μl. © 1994 Wiley‐Liss, Inc.
CD8‐positive adult T‐cell leukemia cells with an integrated defective HTLV‐I genome show a paracrine growth to IL‐2
Authors: Matsushita Kakushi , Arima Naomichi , Hidaka Shiroh , Ohtsubo Hideo , Fujiwara Hiroshi , Fukumori Junko , Tanaka Hiromitsu 
Published: February 2007

AbstractWe describe a 50‐year‐old man with adult T‐cell leukemia complicated by laryngeal tuberculosis whose tumor cells proliferate in response to IL‐2 in a paracrine manner. On admission, the patient's white blood cell count was 17,900/mm3; 73% were abnormal lymphocytes with convoluted nuclel. FACS analysis showed that the tumor cells were CD4‐negative, CD8‐positive T cells. Southern blot analysis of tumor cells revealed integration of a defective HTLV‐I genome lacking gag and pol genes. He was diagnosed with chronic ATL complicated by laryngeal tuberculosis. The primary leukemic cells expressed IL‐2Rα and IL‐2Rβ detected by FACS and Northern blot analysis and showed marked growth in response to exogenously added recombinant IL‐2 in short‐term cultures. Northern blot analysis did not show any IL‐2 mRNA. We have previously demonstrated that primary leukemic cells from some ATL patients grow in response to IL‐2 in an autocrine or paracrine manner. These results suggest that in CD8 ATL, IL‐2 may beinvolved in a paracrine manner. © 1994 Wiley‐Liss, Inc.
Fresh frozen plasma has no beneficial effect on the hemostatic system in children receiving L‐asparaginase
Authors: Halton Jacqueline M. , Mitchell Lesley G. , Vegh Patsy , Eves Marilyn , Andrew Maureen E. 
Published: February 2007

AbstractL‐Asparaginase (ASP), a chemotherapeutic agent used in the treatment of children with acute lymphoblastic leukaemia (ALL), is linked to thromboembolic complications secondary to an acquired deficiency of antithrombin III (ATIII). Fresh frozen plasma (FFP) is used to prevent and/or treat thrombotic complications in these children. However, the effect of FFP on plasma concentrations of ATIII and biochemical markers of activation of coagulation has never been tested. In this study, FFP (20 ml/kg) was administered to eight children with ALL receiving ASP in the consolidation phase of their treatment. Plasma samples were drawn pre‐infusion, and following infusion at 1, 24, and 48 hr. Prior to the FFP infusions, plasma concentrations of prothrombin, fibrinogen, α2‐macroglobulin, heparin cofactor II, protein C, and protein S were similar to levels in healthy children. Only plasma concentrations of ATIII were significantly decreased (0.55 U/ml). Following FFP infusions, there was no statistical or clinically important increase in plasma concentrations of any coagulation protein at any time point. Pre‐infusion plasma concentrations of markers of endogenous thrombin generation (thrombin‐antithrombin III complexes (TAT)) and activation of the fibrinolytic system in response to activation of the coagulation system (D‐dimer levels) were significantly increased. However, FFP had no statistical or clinically important effect on concentrations of these markers. We conclude that FFP administration for the prevention and treatment of acquired ATIII deficiency secondary to ASP has no demonstrable benefit on plasma levels of coagulation proteins and is unlikely to be of clinical benefit. © 1994 Wiley‐Liss, Inc.
HLA‐DR53 protects against thrombotic thrombocytopenic purpura/adult hemolytic uremic syndrome
Authors: Joseph Geetha , Smith Kenneth J. , Hadley Terence J. , Djulbegovic Benjamin , Troup Gary M. , Oldfather John , Barker Robert L. 
Published: February 2007

AbstractClass I and Class II HLA antigens were tested in patients treated for thrombotic thrombocytopenic purpura or hemolytic uremic syndrome (TTP/HUS) to determine whether there is a disease association. Based on the results of a pilot trial, retrospective HLA‐typing of 18 patients with a diagnosis of TTP/HUS and prospective typing of 12 newly diagnosed patients with TTP/HUS were performed. Twenty‐one patients were non‐Hispanic Caucasians, 7 were African‐Americans, and 2 were Hispanic‐Caucasians. Of 30 patients tested, 28 were positive for DR52 (chi‐squared = 5.14, P<0.05), and only two were positive for DR53 compared to 57% of controls (chi‐squared = 18.5, P<0.0005). Diverse DR52 subtypes (DRB3*0101, DRB3*02, and DRB3*0301) were found by oligonucleotide testing in 15 patients, suggesting the association was not with DR52 but with absence of the DR53 antigen. The 2 patients with DR53 were not homozygous. This study suggests that the supertypic antigen, DR53, may govern susceptibility to TTP/HUS, since the relative risk of this disease among DR53 positives is reduced at 0.09 (95% confidence interval, 0.01–0.28). This finding indicates a possible immunogenetic component in the pathogenesis of TTP. © 1994 Wiley‐Liss, Inc.
Effect of low‐dose granulocyte‐macrophage colony‐stimulating factor (LD‐GM‐CSF) on platelet transfusion‐dependent thrombocytopenia
Authors: Vesole David H. , Jagannath Sundar , Glenn Luther D. , Barlogie Bart 
Published: February 2007

AbstractGranulocyte‐macrophage colony‐stimulating factor (GM‐CSF) is a hematopoietic growth factor known to promote the proliferation and differentiation of precursors of granulocytes and monocytes. GM‐CSF at standard doses (125–500 μg/m2) alleviates neutropenia secondary to cytotoxic chemotherapy, myelodysplastic syndromes, and aplastic anemia, but has minimal effect on anemia or thrombocytopenia. GM‐CSF at doses <30 μg/m2 has been reported to improve platelet counts in some patients exhibiting cytopenia related to hematologic disorders such as aplastic anemia and myelodysplastic syndrome. Lowdose GM‐CSF (10–20 μg/m2) was evaluated in 20 patients with transfusion‐dependent thrombocytopenia persisting after myeloablative cytotoxic chemotherapy or with disease‐related cytopenia. Seven patients (35%) responded as defined by a reduction in the platelet transfusion requirements by at least 75%. Low‐dose GM‐CSF did not significantly increase neutrophil counts or decrease red blood cell transfusion requirements. These results indicate that low‐dose GM‐CSF has a thrombopoietic effect in about one‐third of patients with platelet transfusion‐dependent thrombocytopenia which has not been observed at higher doses. © 1994 Wiley‐Liss, Inc.
Inhibitors in hemophilia patients: Current status and management
Authors: Aledort Louis 
Published: February 2007

AbstractThe development of inhibitor antibodies is a long‐standing, well‐documented complication of coagulation factor replacement therapy and is difficult to treat. Previous estimates of 5–15% of patients developing inhibitors may be low, since newer data suggest a wider range of occurrence. Factors that appear to affect inhibitor development include the severity of hemophilia, age, genetics, and, possibly, the type of replacement therapy administered. Recent studies raise the concern that recombinant factor therapies may be associated with more rapid development and higher levels of inhibitors in previously untreated patients. However, results of different studies are often difficult to compare owing to differences in methodology and populations studied. Further studies standardized in design and methods are clearly needed.Management of patients with inhibitors involves control of acute bleeding episodes and, over the long term, Induction of immune tolerance for the coagulation replacement therapy. Many with low or moderate levels of inhibitors may be treated simply by administering higher doses of dotting factor. Other therapies appropriate for those with high levels of inhibitors include porcine F VIII and factor VIII “bypassing” agents, such as recombinant factor VIIa. Long‐term immune tolerance has been achieved through the high‐dose “Bonn” regimen and immunosuppressive regimens such as the “Malmö” method.Although management of inhibitor patients has improved, it still represents a major challenge. Further research is needed to identify which patients will develop inhibitors and tolerance, as well as to develop better methods to manage, reduce, or eliminate inhibitors from these patients. © 1994 Wiley‐Liss, Inc.
Hb E/Hb LeporeHollandia in a family from Bangladesh
Authors: Waye John S. , Eng Barry , Patterson Margaret , Chui David H. K. , Chang Lebe S. , Cogionis Bessie , Poon Annette O. , Olivieri Nancy F. 
Published: February 2007

AbstractWe describe a family from Bangladesh in which three children are compound heterozy‐gotes for Hb E (α2β2, β26Glu Lys) and Hb Lepore (δ‐β fusion gene). PCR amplification and direct nucleotide sequencing established that the fusion gene is Hb LeporeHollandia, with the cross‐over localized to a 40 bp window between codon 22 and IVS‐1 nt 16 of the δ‐ and β‐globin genes. This unusual combination of mutations is associated with a relatively mild clinical phenotype, with all three affected siblings having microcytic anemia of moderate severity without the need for transfusions. © 1994 Wiley‐Liss, Inc.
Acute megakaryoblastic leukemia following transient myeloproliferative disorder in a patient without down syndrome
Authors: Brissette Mark D. , Duval‐Arnould Bertrand J. , Gordon Bruce G. , Cotelingam James D. 
Published: February 2007

AbstractTransient myeloproliferative disorder (TMD) and subsequent acute myeloid leukemia (AML) occur with increased frequency in infants and children with Down syndrome. TMD can also occur in phenotypically normal newborns. We describe the second case of a non‐Down syndrome child with TMD who subsequently developed AML. Trisomy 21 karyotype was restricted to hematopoietic cells in the blood and bone marrow. No other karyotypic abnormalities were found. Leukemic blasts showed megakaryoblastic features with immunophenotyping. This case shows that TMD in a child without Down syndrome may not be entirely benign. Close follow‐up is warranted. © 1994 Wiley‐Liss, Inc.
Antithrombin III kumamoto II; A single mutation at Arg393‐his increased the affinity of antithrombin III for heparin
Authors: Okajima Kenji , Abe Hiroki , Wagatsuma Masako , Okabe Hiroaki , Takatsuki Kiyoshi 
Published: February 2007

AbstractAbnormal antithrombin III (AT III) was found in a 30‐year‐old woman who suffered from recurrent thrombosis during pregnancy and the postpartum period. Among her family members, only her father had recurrent episodes of deep vein thrombosis of the lower extremities, from his youth. The antithrombin and antifactor Xa heparin cofactor activities of the proposita's plasma were 61% and 42% of normal, respectively. The progressive antithrombin and antifactor Xa activities were also decreased to 55% and 58% of normal, respectively. The immunoreactive level of AT III was within the normal range (23.1 mg/dl). Analysis of the proposita's plasma by crossed immunoelectrophoresis in the presence or absence of heparin and by affinity chromatography on heparin‐Sepharose revealed that the proposita's AT III had apparently normal affinity for heparin. Nucleotide sequencing of 7 exons of the proposita's AT III gene amplified by polymerase chain reaction (PCR) disclosed that the second base of codon 393 comprised both G and A, indicating Arg393‐His conversion. The base sequences of exons 1,2,3a, 3b, 4, and 5 were normal, excluding any other mutation. These findings indicated that the proposita's AT III was a variant of AT III at the thrombin binding site and that the proposita was a heterozygote for the abnormality. Heparin affinity of purified abnormal AT III from the proposita's plasma was demonstrated to be increased upon affinity chromatography using heparin‐Sepharose, suggesting that the mutation (Arg393‐His) per se could possibly increase the affinity of antithrombin III for heparin.For this variant AT III (Arg393‐His), the name AT III Kumamoto II is proposed. ©1995 Wiley‐Liss, Inc.
Sickle cell rheology is determined by polymer fraction–not cell morphology
Authors: Hiruma Hiroyuki , Noguchi Constance Tom , Uyesaka Nobuhiro , Hasegawa Setsuo , Blanchette‐Mackie E. Joan , Schechter Alan N. , Rodgers Griffin P. 
Published: February 2007

AbstractSickle cell disease pathophysiology is mediated by acute and chronic impairment of cell flexibility due to the formation of intracellular sickle hemoglobin (Hb S) polymer as cells are partially deoxygenated in the microcirculation. We have recently developed a method to measure the relationship between the formation of intracellular polymerized Hb S and cell filtration. In this study, we have used this method to examine whether sickle cell morphology, independent of Hb S polymer fraction, had an effect on cell rheology. We primarily use sickle trait (AS) and Hb S‐β+‐thalassemia (S‐β+‐thal) erythrocytes with low hemoglobin F levels, which have normal membranes and few or no dense cells, to remove these confounding effects. We find that the relationship between filtration and the percentages of each “type” of morphological deformation of AS erythrocytes was different from that of the S‐β+‐thal erythrocytes. In addition, we find that while the filtration of AS erythrocytes as a function of oxygen saturation was similar, whether measured during deoxygenation or reoxygenation, the relationship between the percentages of each type of deformed erythrocyte and oxygen saturation demonstrated hysteresis during oxygenation‐deoxygenation experiments. Transmission electron microscopy, for both elongated and irregularly shaped cells, showed that similarly distorted cells could have very different amounts and alignment of polymer. These results suggests that cell morphology per se is not strongly related to filtration, whereas calculated intracellular Hb S polymer fraction predicts loss of filtration of AS and S‐β+‐thal erythrocytes well. Measured or calculated polymer fraction values would appear to be a better parameter for the study of sickle cell disease pathophysiology and response to treatment than cell morphology studies. ©1995 Wiley‐Liss, Inc.
Unexpected hepatotoxicity after priming and treatment with molgramostim (rhGM‐CSF) in acute myeloid leukemia during induction chemotherapy
Authors: Hansen Per Boye , Johnsen Hans E. , Lund Jens Otto , Hansen Mogens Sandbjerg , Hansen Niels Ebbe 
Published: February 2007

AbstractThe effect of supplementing induction chemotherapy with recombinant human granulocyte‐macrophage colony‐stimulating factor (rhGM‐CSF) was studied in a randomized trial of 18 patients with acute myeloid leukemia (AML). Ten patients received rhGM‐CSF, starting on day one to three before chemotherapy and continued for a maximum of 21 days after the start of induction treatment. Unexpected adverse effects of rhGM‐CSF and chemotherapy combination included a transient decline in plasma coagulation factors II, VII, and X (5 of 5 patients) and an increased transcapillary escape rate of albumin (in 3 of 3 patients tested). The decline in coagulation factors was prevented in subsequent patients by prophylactic treatment with vitamin K. Although the small number of patients studied may not allow a definite conclusion, caution with regard to liver function should be shown in combining rhGM‐CSF with intensive chemotherapy. ©1995 Wiley‐Liss, Inc.
Role of interferon alpha‐2a in the treatment of polycythemia vera
Authors: Foa Paolo , Massaro Paolo , Ribera Sonia , Lurlo Alessandra , Mezzanotte Carmen , Giandalia Daniela , Maiolo Anna Teresa 
Published: February 2007

AbstractWe studied the effects of recombinant interferon alpha‐2a (IFN‐alpha) in 36 patients with polycythemia vera (PV) previously treated with phlebotomy and/or conventional cytostatic agents. In each patient, after at least 2 months of discontinuation of any cytotoxic therapy, the hematocrit (Hmt) was first brought to normal value by phlebotomy; IFN‐alpha treatment was then begun at a starting dose of 3,000,000 IU s. c. three times a week. Response to treatment, which was assessed monthly, was defined as persistent normalization of Hmt without concomitant phlebotomy; in non‐responsive patients the initial IFN‐alpha weekly dosage was progressively increased. Twenty patients were responsive with a median duration of response of 7 months (range 2‐25+ months); out of these, 7 patients are still under treatment and responsive at 13+, 17+, 20+, 22+, 23+, 25+, 25+ months. These findings indicate that a cohort, although small, of patients with PV (19.4%) are persistently sensitive to IFN‐alpha; in this subset of patients, this cytokine can therefore provide a useful treatment option, since, contrary to conventional therapeutic approaches such as radioactive phosphorus, cytostatic agents, or phlebotomy, IFN‐alpha is devoid of harmful side effects. ©1995 Wiley‐Liss, Inc.
Initiation codon mutation (ATG → ATA) of the β‐globin gene causing β‐thalassemia in a Swedish family
Authors: Landin Britta , Rudolphi Olle , Ek BÖRje 
Published: February 2007

AbstractAn initiation codon mutation ATG → ATA of the β‐globin gene was found in seven members of three generations of a family living in northern Sweden. This mutation, which has not previously been described, changes the initiation codon for methionine into a codon for isoleucine and will then result in a β°‐thalassemic phenotype. The affected family members all present hematological findings typical for β‐thalassemic trait, with slight anemia, marked microcytosis, and increased levels of Hb A2. ©1995 Wiley‐Liss, Inc.
Fibrinogen/fibrin degradation products and D‐dimer in clinical practice: Interpretation of discrepant results
Authors: Sato Naoaki , Takahashi Hoyu , Shibata Akria 
Published: February 2007

AbstractIn clinical practice, occasionally some patients show dissociated values of fibrinogen / fibrin degradation products (FDP) and D‐dimer (cross‐linked fibrin degradation products). In an attempt to assess the frequency, clinical backgrounds, and hemostatic states of these cases, FDP and D‐dimer were simultaneously measured together with other hemostatic parameters in 371 samples from patients with various diseases. As a whole, FDP values were positively correlated with D‐dimer values (r = 0.871, P < 0.0001), and both were elevated in parallel with the progress of activation of blood coagulation and fibrinolysis. However, in patients with elevated FDP and/or D‐dimer, 11.5% of samples showed relatively lower D‐dimer values than those expected from FDP levels, and these were regarded as an apparently dissociated group. In the dissociated group, activation of coagulation and fibrinolysis occurred to a lesser extent than others. Analysis of these samples suggested that the possible reasons for the dissociation bet ween FDP and D‐dimer values were accelerated fibrinogenolysis with or without secondary fibrinolysis, accelerated fibrinogenolysis by non‐plasmic proteinases, elevated soluble fibrin, and possibly false‐positive FDP levels due to unclottable fibrinogen remaining in the serum samples. In practice, simultaneous measurements of FDP and D‐dimer are useful for more accurate estimation of hyperfibrinolytic states. ©1995 Wiley‐Liss, Inc.
Morphological, ultrastructural, and genetic characterization of an unusual T‐cell lymphoma in a patient with sinus histiocytosis with massive lymphadenopathy
Authors: Koduru P. R. K. , Susin M. , Kolitz J. E. , Sonl M. , Teichberg S. , Siques M. J. , Sun T. , Amorosi E. , Budman D. R. 
Published: February 2007

AbstractSinus histiocytosis with massive lymphadenopathy (SHML) is a rare benign disease of unknown etiology. It is rarely associated with malignant lymphoma. This report documents the first case of a T‐cell lymphoma, which developed in a patient with a 10‐year history of SHML. The disease was complicated by hypereosinophilia and massive retroperitoneal lymphadenopathy. Histological examination of a cervical lymph node biopsy during the terminal phase identified a lymphoma composed of cells with morphological plasmacytoid features. Ultrastructurally, the tumor cells showed poorly developed cytoplasm, nuclei with peripheral chromatin clumping, and inconspicuous nucleoli. Cytogenetic studies showed two related clones. On immunohistochemical staining tumor cells were positive with monoclonal antibodies (mAb) CD3 and CD45RO. Southern blotting analysis identified clonal rearrangements in the T‐cell receptor (TCR) alpha, beta and gamma genes. Thus, T‐cell lineage of the tumor cells was established. In situ hybridization of interleukin‐2 (IL‐2) and interleukin‐5 (IL‐5) cDNA probes on tissue sections identified the synthesis of IL‐5 by the eosinophils, suggesting an autocrine pathway of eosinophilopoiesis leading to hypereosinophilia in this patient. ©1995 Wiley‐Liss, Inc.