bluuebunny/crossref_metadata_2025_split huggingface dataset:
Apparent response of refractory post‐transfusion purpura to splenectomy
Authors: Cunningham C. Casey , Lind Stuart E. 
Published: February 2007

AbstractPost transfusion purpura (PTP) is a rare disorder characterized by the abrupt onset of severe thrombocytopenia following transfusion. A patient with PTP and massive bleeding was refractory to corticosteroids, gamma globulin, and plasma exchange but developed an immediate and sustained rise in platelet count following splenectomy. Splenectomy may be a useful therapeutic modality in patients with refractory PTP.
Evaluation of factor VIII pharmacokinetics in hemophilia‐A subjects undergoing surgery and description of a nomogram for dosing calculations
Authors: Longo G. , Messori A. , Morfini M. , Baudo F. , Ciavarella N. , Cinotti S. , Filimberti E. , Giustarini G. , Molinari A. C. , Ferrini Pierluigi Rossi 
Published: February 2007

AbstractThe pharmacokinetics of factor VIII were studied in a series of 20 hemophilia‐A patients undergoing surgery. Regardless of the type of operation, elimination of factor VIII was shown to be increased only in ten cases (50%) during the post‐operative period. In this subgroup of patients, factor VIII half‐life, measured immediately after surgery, was considerably shorter (mean = 9.6 hr, n = 10) than that determined in the same individual during the late operative period (mean = 17.8 hr, n = 10). These findings indicate that identification of patients with increased postoperative consumption of factor VIII can be of value in reducing the risk of hemorrhage in these subjects and in exposing other subjects with no postoperative increase in factor VIII clearance to less of the deficient factor. Data from 20 subjects were analyzed to construct a nomogram allowing individualized prediction of factor VIII dosing requirements. The nomogram, which is based on the “single point after a single dose” method, uses a value of factor VIII concentration measured at 10 hr after preoperative loading dose to predict the regimen producing the desired average steady‐state concentration of factor VIII (30, 60, or 90 units/dl).
Spontaneous platelet aggregation in type IIB tampa von willebrand disease is inhibited by the 52/48‐kDa fragment of normal von willebrand factor, which contains the GPIb binding domain
Authors: Saba Hussain I. , Fujimura Yoshihiro , Saba Sabiha R. , Bruce Anita C. , Morelli Genevieve A. , Ruggeri Zaverio M. , Zimmerman Theodore S. 
Published: February 2007

AbstractThe association of Type IIB von Willebrand disease (vWD) with chronic persistent thrombocytopenia and spontaneous platelet aggregation has recently been recognized. It has been shown that IIB von Willebrand factor (vWF) can initiate platelet aggregation by binding to the platelet glycoprotein (GP) lb receptor and inducing exposure of the Gpllb/llla fibrinogen receptor. In this study we demonstrate the increased binding of Type IIB Tampa vWF with normal platelets when compared with nonthrombocytopenic Type IIB vWF. Studies further demonstrate that spontaneous platelet aggregation initiated by IIB Tampa vWF can be blocked by a 52/48‐kDa fragment of normal vWF, which contains the binding domain.
Adult‐onset cyclic bicytopenia: A case report and review of treatment of cyclic hematopoiesis
Authors: Tefferi Ayalew , Solberg Lawrence A. , Petitt Robert M. , Willis Larry G. 
Published: February 2007

AbstractA unique case of adult‐onset synchronous cyclic neutropenia and thrombocytopenia occurring at six‐week intervals is presented. Periods of cytopenia were associated with fever, myalgias, gastrointestinal symptoms, and mild mucocutaneous bleeding. Alternate‐day steroid treatment failed to correct the periodic fluctuations in peripheral blood counts but ameliorated symptoms during cytopenia. The treatment of cyclic hematopoiesis is reviewed.
Factor VIII inhibitor in insulin‐dependent diabetes mellitus
Authors: Wajima Takeshi , Hall Gregory 
Published: February 2007

AbstractAcquired factor VIII inhibitor was found in a 69‐year‐old white male with insulin‐dependent diabetes mellitus. He presented with left lower abdominal pain and hematoma after a fall. Preoperative hemostasis studies were normal except for prolonged aPTT. Prolonged aPTT was not corrected by 1:1 mixture with normal fresh plasma and incubation showed further prolongation with time. Factor VIII:c was 3.5%. The inhibitor titer was 7.5 Bethesda units. The possible mechanism causing antibody to factor VIII was postulated to be an autoimmune process and/or increased immunogenicity owing to glycosy‐lation of factor VIII coagulant protein.
Red cell ferritin and iron overload in heterozygous beta‐thalassemia
Authors: van der Weyden Martin B. , Fong Hubert , Hallam Lynnette J. , Harrison Christine 
Published: February 2007

AbstractRed cell ferritin was evaluated in 101 individuals with heterozygous beta‐thalassemia to determine its clinical utility as an index for iron deficiency or overload in these subjects. The mean red cell ferritin for the total population was elevated threefold and showed a significant correlation with transferrin saturation, plasma ferritin, and HbA2 levels. Five of six subjects with reduced red cell ferritin had associated iron deficiency; a further five had iron deficiency and normal red cell ferritin. Normal red cell ferritin occurred in 51 subjects, and 44 had increased values. In the elevated red cell ferritin group, 21 individuals had associated normal plasma ferritin, and 23 had increased plasma ferritin. Only in the latter group was red cell ferritin significantly correlated with transferrin saturation and plasma ferritin. Ten individuals had a red cell ferritin ≤ 150 attogram/cell, and liver biopsy performed in four showed grades II to IV iron overload. A clinical feature of subjects with both increased red cell and plasma ferritin levels was a high incidence of inappropriate iron administration. These findings suggest that red cell ferritin, particularly when combined with plasma ferritin, is a useful parameter for determining potential iron overload in individuals with heterozygous beta‐thalassemia.
HLA antibody formation within the HLA‐A1 crossreactive group in multitransfused platelet recipients
Authors: Macpherson Bruce R. 
Published: February 2007

AbstractThe formation of intragroup antibodies, HLA antibodies directed against antigens in the same crossreactive group (CREG) as those of the serum donor, may be an important cause of transfusion failures in patients receiving HLA‐matched platelets. Twenty‐two patients whose HLA types included at least one antigen in the HLA‐A1 CREG were studied. Of the ten patients who formed HLA antibodies, six produced antibodies that reacted with one or more antigens in the HLA‐A1 CREG. Five of 12 patients whose HLA types included HLA‐A3 formed antibodies directed against HLA‐A1‐10‐11 or HLA‐A1‐10. In contrast, only one of ten individuals whose phenotypes included HLA‐A1, HLA‐A11, or both produced anti‐HLA‐A3. Eleven incompatible retrospective crossmatches were observed in recipients of HLA‐matched platelets attributable to intragroup antibodies. Patients receiving incompatible platelets had unsatisfactory post‐transfusion platelet count increments. It is concluded that intragroup antibodies, such as those directed against antigens in the HLA‐A1 CREG, are an important cause of platelet transfusion failures in patients requiring long‐term platelet transfusion support. These antibodies can be identified by routinely screening recipient sera for HLA antibodies and performing retrospective crossmatches using the lymphocytotoxicity technique.
Hybrid acute leukemia in an HIV‐antibody‐positive patient
Authors: Gold Jay E. , Babu Arvid , Penchaszadeh Victor , Castella Antonio , Ghali Violette , Zalusky Ralph 
Published: February 2007

AbstractAlthough the great majority of acute leukemias have been designated as being of lymphocytic or myelocytic origin, recent reports have described elements of both in some patients. We describe here the first case of hybrid acute leukemia in an HIV‐antibody‐positive patient as well as the first hybrid involving B‐cell (Burkitt) acute lymphocytic leukemia and acute myelomonocytic leukemia proven by cytochemical, immunologic, and cytogenetic methods. This case illustrates the increasingly complex difficulties in the diagnosis and treatment of AIDS‐related malignancies.
Long‐term effect of splenectomy on transfusion requirements in thalassemia major
Authors: Cohen Alan , Gayer Robin , Mizanin Jeannie 
Published: February 2007

AbstractSplenectomy reduces transfusion requirements in the first year after surgery in patients with thalassemia major and hypersplenism. To determine whether this response is maintained, we have studied transfusion requirements in 16 patients with thalassemia major for 2–17 years after splenectomy. Transfusion requirements remained stable (mean yearly change ‐0.1%) after the predictable fall in the first year after surgery. The mean change between the first postoperative year and the most recent year was ‐7 ml/kg. Transfusion requirements and the magnitude of change from year to year were unrelated to the time since splenectomy. These studies indicate that the effect of splenectomy on transfusion requirements is long‐lasting and that large variations in annual transfusion requirements after splenectomy should prompt a search for accessory spleens or other causes of red cell destruction.
B‐cell chronic lymphocytic leukaemia: Prognostic value of the immunophenotype and the clinico‐haematological features
Authors: Orfao Alberto , Gonzalez Marcos , Fernando Miguel J. San , Rios Agustin , Canizo M. Consuelo , Hernandez Jose , Maricato M. Lourdes , Borrasca Antonio Lopez 
Published: February 2007

AbstractSixty‐two previously untreated patients with B‐cell chronic lymphocytic leukaemia were analysed to study the prognostic value of both the immunologic phenotype and the clinicobiologic characteristics. Univariate studies showed that none of the immunological markers analysed, sheep‐rosette, mouse‐rosette, slg, and HLA/DR, CD20, FMC7, CD5, and CD9 antigens, had a significant influence on survival. On the other hand, several clinical and haematological characteristics were identified as being associated with survival: 1) clinical features—presence of lymphadenopathies (P< .05) and hepatomegaly and/or splenomegaly (P< .04); 2) haematologic parameters—presence of anaemia and/or thrombopenia (P< .05), the absolute peripheral blood lymphocyte count (P< .03), and the presence of hypogammaglobulinemia (P< .08); 3) biochemical parameters—serum uric acid (P< .03); and 4) bone marrow histopathological features—biopsy pattern (P< .04) and the percentage of lymphocytes in bone marrow aspirate (P< .03). Both the Rai staging and the International Workshop on CLL staging systems were effective in identifying groups of patients with significantly different prognoses (P< .05). Multivariate regression analysis demonstrated that the combination of three clinicopathologic characteristics (bone marrow histopathologic pattern, absolute peripheral blood lymphocyte count, and the presence or not of hypogammaglobulinaemia) had the strongest predictive relationship with survival time.In summary, our findings show that the clinicobiological and anatomopathologic parameters have much more prognostic relevance than the immunological markers analysed in the present study.
Abnormal fibrinolysis in healthy male cigarette smokers: Role of plasminogen activator inhibitors
Authors: Haire William D. , Goldsmith Jonathan C. , Rasmussen Julie 
Published: February 2007

AbstractThe extrinsic fibrinolytic system and its response to cigarette smoking was studied in five healthy male smokers 35–45 years old. Tissue plasminogen activator (t‐PA) release in response to venous occlusion was intact both at 8:00 A.M. and 3:00 P.M. Acutely smoking two cigarettes neither stimulated fibrinolysis nor changed levels of t‐PA or plasminogen activator inhibitors. Functional plasminogen activator inhibitor (PA‐I) levels and euglobulin lysis times were higher in the smoking group than in a control group matched for age, sex, and body mass. Antigenic levels of PA‐I 1, the PA‐I derived from vascular endothelial cells and platelets, were similar in both groups. While smoking did not acutely alter fibrinolysis in chronic smokers, these individuals had a high frequency of abnormal fibrinolysis characterized by high levels of PA‐I activity. This abnormality is due to either high specific activity of PA‐I 1 or to the presence of other antigenically distinct plasminogen activator inhibitors. Abnormal firbinolysis may be one mechanism contributing to the thrombotic diathesis of cigarette smokers.
Emergency caesarian section and symptomatic immune thrombocytopenic purpura
Authors: Wood Lucille , Jacobs Peter 
Published: February 2007

AbstractA 24‐year‐old woman presented in labour with profound purpuric bleeding due to idiopathic immune thrombocytopenia. She was assessed as requiring immediate caesarian section for cephalopelvic disproportion and foetal distress. In view of her platelet count of 21 × 109/L she was prepared with intravenous methylprednisolone, isovolaemic plasma exchange, and infusion of 400 mg/kg of gammaglobulin and 5 × 1011 allogeneic platelets. Six hours after commencing the procedure, at a time when her platelet count was 97 × 119/L, she went uneventful operation and with no further therapy this level subsequently reached 175 × 109/L, at which time an uneventful operation was performed. The mother and her normal, full‐term infant have been discharged; both are well and at follow‐up have normal platelet counts. This experience illustrates that major surgery can safely be undertaken in severely thrombocytopenic patients, even as an emergency procedure, using this regimen.
Protein S and C antigen levels in proteinuric patients: Dependence on type of glomerular pathology
Authors: Allon Michael , Soffer Oved , Evatt Bruce L. , Hixon Gwen , Wideman Carol S. 
Published: February 2007

AbstractThe cause of the thrombotic tendency in nephrotic patients is unknown. Recent reports of thrombotic complications in patients with deficiencies of protein C or protein S (natural inhibitors of coagulation) have raised the possibility that decreased levels of these proteins may play a role in the hypercoagulable state of nephrotic patients. We measured the levels of protein C, total protein S, and free protein S antigens in 42 patients (21 nephrotic and 21 non‐nephrotic) with one of four types of glomerular pathology: diabetic nephropathy (DM), focal glomerular sclerosis (FGS), membranous glomerulonephritis (MGN), and chronic renal failure due to hypertension (CRF). Protein C and total protein S antigen levels were significantly higher in FGS and MGN than they were in DM or CRF. Free protein S levels were lower in DM than they were in MGN. Protein C, total protein S, and free protein S levels did not significantly correlate with either serum albumin or degree of proteinuria. The mean levels of the three proteins did not differ between nephrotic and non‐nephrotic patients. Free protein S and protein C were, however, significantly correlated (P < .005 and P < .002, respectively) with the type of glomerular pathology, independent of differences in age, sex, serum albumin, or degree of proteinuria. These data suggest that abnormalities of free protein S and protein C are related to the nature of the underlying renal disease, rather than to the degree of proteinuria.
Heparin cofactor II in adults and infants with thrombosis and DIC
Authors: Chuansumrit Ampaiwan , Manco‐Johnson Marilyn J. , Hathaway William E. 
Published: February 2007

AbstractHeparin cofactor II (HC II) was measured by a chromogenic activity assay in normal preterm infants (gestational age, 28–36 weeks; n = 17; 29% ± 11.5 [mean ± 1 SD], range 11–51), normal full‐term infants (n = 18; 49% ± 6.6 [mean ± 1 SD], range 36–58), and normal adults (n = 38; 101% ± 14 [mean ± 1 SD], range 73–130). Normal children attained adult levels at approximately 5 to 7 months of age. The lower values in preterm and term infants most likely reflect immature liver function.Neither adults and children with a history of thrombosis with prior negative evaluation (n = 74), patients with documented protein C and protein S deficiency (n = 4), nor sick infants without evidence of consumptive coagulopathy (n = 15) had significantly lower levels of HC II activity. Infants with disseminated intravascular coagulation (n = 2) had strikingly lower levels of HC II activity.
Chronic myeloid leukemia associated with pure red cell aplasia and terminating in promyelocytic transformation
Authors: Mijovic Aleksandar , Rolovic Zoran , Novak Angelina , Biljanovic‐Paunovic Lidija , Tomin Dragica 
Published: February 2007

AbstractAfter intermittent treatment with busulphan over a 7‐year period for chronic myeloid leukemia (CML) in chronic phase, a 39‐year‐old female developed leukocytosis in association with pure red cell aplasia (PRCA). Bone marrow examination confirmed erythroid aplasia, and culture revealed a total absence of erythroid progenitor cells. The patient then was treated with azathioprine, corticosteroids, cyclophosphamide, plasma exchange, and cyclosporin A, but she remained erythroblastopenic and transfusion dependent for more than a year, at which time a promyelocytic transformation supervened. The authors propose that this sequence of events, hitherto unreported, is a manifestation of the multistep progression of CML.
Hemoglobin synthesis in BFU‐E colonies derived from transfused blood in a sickle cell anemia patient
Authors: Bhaumik Kabita 
Published: February 2007

AbstractIn an in vitro progenitor cell culture system, a significant amount of normal hemoglobin (Hb A) synthesis was observed in a transfused sickle cell anemia (SS) patient, indicating the contribution of stem cells obtained from the normal donor's blood cells. As this study was done long after the last blood transfusion (> 100 days), the survival of the donor's stem cells would indicate their repopulating the recipient's bone marrow. On extending the study to a few other transfused SS patients who had received multiple blood transfusions at frequent intervals, no such synthesis of Hb A was observed. A loss of repopulating ability of the donor's stem cells with frequent transfusions at short intervals is suggested as a possible explanation, with a gradual development of immune response against these cells as a second possibility. The differences in the survival of the donor's stem cells would thus appear to have some reflection on the frequency of blood transfusions required by an individual patient.
Steroid‐responsive pure red cell aplasia associated with natural killer cell lymphocytosis
Authors: Tefferi Ayalew , Windebank Kevin P. , Veeder Michael H. , Kiely Joseph M. 
Published: February 2007

AbstractA patient with pure red cell aplasia and expansion of the peripheral blood natural killer (NK) cell population is described. Despite normal absolute and differential leukocyte counts, NK cells were increased at diagnosis and at relapse. Furthermore, these cells were not morphologically recognizable on the peripheral blood smear examination. A favorable clinical response to glucocorticoid therapy was accompanied by a decrease in NK cells.
Agranulocytosis associated with “mexican aspirin” (dipyrone): Evidence for an autoimmune mechanism affecting multipotential hematopoietic progenitors
Authors: Hargis J. B. , La Russa V. F. , Redmond J. , Kessler S. W. , Wright D. G. 
Published: February 2007

AbstractA case of acute, transient agranulocytosis and thrombocytopenia associated with ingestion of dipyrone is reported. This once widely used analgesic, which is now banned in the United States, was obtained by the patient as “aspirin” while traveling in Mexico. Studies of the effects of this patient's serum on purified CD34+ marrow cells, which were highly enriched for hematopoietic progenitors, showed not only a drug‐dependent suppression of the in vitro growth of myeloid progenitors, as has been reported previously, but also a drug‐dependent suppression of primitive multipotential progenitors (CFU‐Mix) and erythroid progenitors (BFU‐E). These findings indicate that autoimmune, antibody‐hapten interactions which have been reported to occur in dipyrone‐ and aminopyrine‐induced agranulocytosis are not restricted to the neutrophil lineage.
Platelet activation during pain crisis in sickle cell anemia patients
Authors: Beurling‐Harbury Christina , Schade Stanley G. 
Published: February 2007

AbstractPlatelet activation at sites of enmeshed sickled red cells in the microcirculation may contribute to platelet plug formation and microinfarction in sickle cell anemia. To test this hypothesis platelets from 116 sickle cell anemia patients free of crisis, 32 patients with crisis, 16 convalescents within 1 week of crisis, and 180 normal controls were studied. Platelets store 90% of their ADP in dense secretory granules. During activation ADP is secreted and permanently lost from the cell. This leads to a decrease in cellular ADP concentration and a sharp rise in the ATP/ADP ratio. ATP and ADP were ethanol‐extracted from platelet‐rich plasma, measured in the luciferase‐luciferin assay and expressed in nmoles per 108 cells. No adenine nucleotide differences were found in platelets from patients free of crisis compared with normal controls. The ADP concentration of platelets from patients in crisis was significantly lowered, indicating that in vivo platelet secretion of ADP had occurred. Total and released ADP was decreased from 2.69 to 1.66, and from 1.90 to 1.21 respectively, and the total ATP/ADP ratio was increased from 1.85 to 2.84 (P < 0.001). ADP stores in platelets from convalescents were significantly different from sickle controls (P < 0.001) but were less abnormal than ADP stores in platelets from crisis patients (P < 0.01), indicating recovery. Total and released ADP was decreased to 1.97 and 1.31 respectively, and the ATP/ADP ratio was increased to 2.38. Platelets from patients in crisis were able to release their remaining granular ADP in response to thrombin as effectively as normal platelets. Thus significant platelet activation with ADP release occurs during acute sickle pain crisis. This might contribute to platelet plug formation and microvascular obstruction.
Positional effect of cis/trans alpha globin gene deletions on the formation of “H” bodies
Authors: Thompson C. C. , Ali M. A. M. , Boyadjian S. , Vacovsky M. 
Published: February 2007

AbstractNormal individuals have four AaL‐globin genes, two on each member of the chromosome 16 pair (αα/αα). The α‐thalassemia trait phenotype associated with deletions of two α‐genes can be either on the same chromosome, the cis type (αα/—), or on opposite chromosomes, the trans type (α‐/α‐). Traditionally, the observation on vitally stained smears of occasional cells containing “H” bodies has been used as an important diagnostic criterion for α‐thalassemia trait. These “H” bodies are thought to be precipitated β tetramers because of the presence of excess β‐globin chains. Our study in patients with various α‐genotypes indicates that normal subjects (αα/αα) and patients with silent α‐thalassemia trait (αα/α‐) generally have no “H” bodies. However, patients with the two‐gene deletion of the cis type α‐thalassemia (αα/—) show the occasional “H” body, and those with Hb “H” disease (α‐/—or αcs‐/—) show many such bodies. On the other hand, patients with two‐gene deletion of the trans type (α‐/α‐) do not show “H” bodies.The number of “H” bodies found does not appear to correlate directly with the degree of imbalance in α‐ and β‐chain production among the various α‐genotypes examined. The chemical nature of “H” bodies is discussed, and an alternative hypothesis that embryonic ζ chains expressed in the cis type but not in the trans type of α‐thalassemia are involved in the formation of “H” bodies is proposed.