bluuebunny/crossref_metadata_2025_split huggingface dataset:
1‐deamino‐8‐D‐arginine vasopressin (DCAVP) increases platelet membrane expression of glycoprotein Ib in patients with disorders of platelet function and after cardiopulmonary bypass
Authors: Sloand Elaine M. , Alyono David , Klein Harvey G. , Chang Ping , Yu Monica , Lightfoot Fred G. , Kessler Craig 
Published: February 2007

Abstract1‐deamino‐8‐D‐Arginine vasopressin (DDAVP) shortens the bleeding time in some patients with platelet dysfunction and decreases blood loss in some cardiopulmonary bypass patients. We studied platelet membrane glycoproteins in patients with von Willebrand disease (vWD), disorders of platelet function, and in cardiopulmonary bypass patients after infusion of 0.3 μg/kg of DDAVP. Platelets from 8 cardiopulmonary bypass patients, receiving DDAVP immediately after surgery, were compared to those of 14 patients not receiving DDAVP. We also studied 12 patients with vWD, and 8 patients with platelet dysfunction receiving DDAVP. Fixed platelets, stained with monoclonal fluorescein (FITC)‐labeled antibodies directed against GPIb (CD42b antigen), GPIb/IX, GPIIb/IIIa (CD41a antigen), CD63 antigen (a platelet activation protein), and P‐selectin (CD62 antigen) were studied by flow cytometry. Binding of CD42b monoclonal antibody (MoAb) and anti‐GPIb/IX to platelets from both groups of bypass patients increased during the 18–20 hr after surgery, but the group receiving DDAVP showed the greater increase (P = 0.032). Platelets from patients receiving DDAVP for vWD or for platelet dysfunction, had increases in CD42b MoAb and anti‐GPIb/IX binding (P < 0.01) that coincided with shortening of their bleeding time. No changes were seen in binding of other antibodies. When platelets from normal donors were incubated with DDAVP for 20 hr, there were increases in platelet surface CD42b MoAb binding, while immunogold‐stained transmission electron micrographs of permeabilized platelets demonstrated decreases in cytoplasmic CD42b MoAb binding. DDAVP increases platelet membrane GPIb expression in a variety of patients and may account for improvement in hemostasis seen in some studies. Redistribution of GPIb from the cytoplasm to the membrane may account for this increased expression. © 1994 Wiley‐Liss, Inc.
Increased thrombosis incidence in a family with an inherited protein S deficiency and a high oxygen affinity hemoglobin variant
Authors: Berruyer M. , Francina A. , Ffrench P. , Negrier C. , Boneu B. , Dechavanne M. 
Published: February 2007

AbstractInherited protein S deficiency and the presence of a rare high oxygen affinity hemoglobin variant: Hb Rainier [β 145 (HC2) Tyr → Cys] were found in a family. Among 16 studied members, nine were found as carriers of protein S deficiency (type I with decrease of total, free, and activity levels). Six subjects carried the high‐affinity hemoglobin variant, which displayed an increase of blood viscosity. Four members combined both abnormalities. Three had thrombotic accidents before the age of 30. We suggest the combination of protein S deficiency and the presence of this hemoglobin variant can lead to a severe primary hypercoagulable state with pathological consequences compared to each genetic defect alone. © 1994 Wiley‐Liss, Inc.
Gray platelet syndrome with splenomegaly and signs of extramedullary hematopoiesis: A case report with review of the literature
Authors: Jantunen Esa , Hänninen Auli , Naukkarinen Anita , Vornanen Martine , Lahtinen Reino 
Published: February 2007

AbstractGray platelet syndrome (GPS) is a rare bleeding disorder characterized by thrombocytopenia, agranular gray platelets in blood films, and almost total absence of platelet alpha‐granules and their constituents. We describe here a rare case of GPS with myelofibrosis and splenomegaly indicating extramedullary hematopoiesis. Splenectomy was followed by normalization of platelet count but the bleeding diathesis continued. Based on a follow‐up of more than 15 years, the slight myelofibrosis in our patient seems to be non‐progressive. We also summarize the major clinical and laboratory features of previously published cases of GPS in order to obtain more comprehensive understanding of this rare disorder. From the clinical point of view, the bleeding tendency in this syndrome generally varies from mild to moderate, and no specific treatment is usually needed. Careful examination of peripheral blood films is necessary for the diagnosis of this rare syndrome. © 1994 Wiley‐Liss, Inc.
Promace day 1/MOPP day 8 chemotherapy for prolymphocytic leukemia: A case report of prolonged survival
Authors: Yuen Benjamin H. , Godwin John E. 
Published: February 2007

AbstractThe treatment result of a case of de novo B‐cell prolymphocytic leukemia (B‐PLL) is described. The diagnosis was established with histologic, ultrastructural and immunologic studies. The patient was treated with chemotherapy using a total of seven cycles of ProMACE Day 1/MOPP Day 8 lymphoma regimen. Complete remission was achieved after 5 cycles of chemotherapy. The patient remains disease free after a 7‐year follow up. This is the first case of PLL being treated with such a regimen and the only case reported achieved such prolonged survival. Results of other treatment modalities are reviewed. © 1994 Wiley‐Liss, Inc.
Hemoglobinopathies in the dogon country: Presence of βs, βc, and δa'genes
Authors: Ducrocq Rolande , Bennani Mohcine , Bellis Gil , Baby Mounirou , Traore Kader , Nagel Ronald L. , Krishnamoorthy Rajagopal , Chaventre Andre 
Published: February 2007

AbstractThe population of the Dogon, located in Mali, is divided in an endogamic Noble class and two endogamic servant castes (Tanners and Blacksmiths). We find that the polymorphic frequencies of βc, βs, and, unexpectedly, a mutation of the δ‐chain (δA'), are geographically (valley vs. plateau) as well as social status dependent. © 1994 Wiley‐Liss, Inc.
Karyotype in myelodysplastic syndromes: Relations to morphology, clinical evolution, and survival
Authors: Bernasconi P. , Alessandrino E. P. , Boni M. , Bonfichi M. , Morra E. , Lazzarino M. , Campagnoli C. , Astori C. 
Published: February 2007

AbstractOne hundred eighty‐eight unselected consecutive patients with “de novo” myelodysplastic syndrome (MDS) were studied cytogenetically. They were subclassified as 4 refractory anemia with ringed sideroblasts (RARS), 67 refractory anemia (RA), 58 refractory anemia with excess of blasts (RAEB), 40 RAEB in transformation (RAEB‐t), and 19 chronic myelomonocytic leukemia (CMML). The overall incidence of chromosome abnormalities was 69%. The RAEB and RAEB‐t patients showed karyotypic changes, more often than RA and CMML (76% and 100% vs. 56% and 42%, respectively). The most frequent single anomaly was del(5)(q13‐q22q33) (22 cases), followed by monosomy 7 or del 7q (11 cases), del(11) (q14q23) (8 cases), trisomy 8 (4 cases). Complex karyotypes (defined by the presence of three or more structural or numerical abnormalities) were detected in 33 patients. With regard to the FAB classification, del (5)(q13q33) was associated with RA, and complex rearrangements with RAEB and RAEB‐t. Leukemic transformation occurred in 66 patients (46%), none with a normal karyotype or del(11)(q14q23) as single abnormality. In patients carrying 5q‐ alone, acute evolution correlated with proximal breakpoint localization, being found in no case with del(5)(q13q33) but in three out of four cases with del(5)(q22q33). Acute leukemia (AL) progression happened in all cases with complex rearrangements and monosomy 7 or del(7q). Two of the four trisomy eight patients evolved in AL. By using the Cox proportional hazard regression analysis it was demonstrated that the karyotype abnormality was a significant predictor of leukemic transformation (P < 0.001). Patients with abnormal karyotypes without complex abnormalities had a survival (median survival 12 months) shorter than that of cases with only normal metaphases (median 83 months) (P < 0.001); patients with a mixture of normal/abnormal metaphases had a median survival of 31 months. The median survival for complex karyotypes was 7 months. Among cases with single defects, del(5)(q13q33) showed the best survival (64 months), mono‐ somy 7 and del (7q) the worst (7 months) (P < 0.001). © 1994 Wiley‐Liss, Inc.
Hepatitis C virus infection in patients with leukemia
Authors: Fujii Yasuhiko , Kaku Kohei , Tanaka Masahisa , Yosizaki Miki , Kaneko Toshio , Matumoto Noboru 
Published: February 2007

AbstractWe have studied 30 patients with acute leukemia by the second‐generation assay for antibodies to hepatitis C virus (HCV) to determine the incidence of HCV infection and the impact of anti‐HCV positivity on liver disease. After a complete remission, 21/30 (70%) patients were anti‐HCV‐positive. During chemotherapy the anti‐HCV‐positive patients had more severe liver disease than the anti‐HCV‐negative patients, and they had a higher incidence of chronic hepatitis (13/21; 62% vs. 1/9; 11%, P < 0.01). During subsequent follow‐up, 15/30 (50%) patients relapsed and 15/30 (50%) patients completed the chemotherapy protocols. After a relapse 12/15 (80%) patients were anti‐HCV‐positive and they had more severe liver disease than the anti‐HCV‐negative patients. Among the patients who completed chemotherapy (n = 15), biochemical evidence of chronic hepatitis was found in 9/9 (100%) anti‐HCV‐positive, and 2/6 (33%) anti‐HCV‐negative cases during off‐therapy follow‐up after therapy‐withdrawal (P < 0.05). These results indicate that HCV plays an important role in the etiology of chronic hepatitis which could worsen the final prognosis of successfully treated patients with leukemia. © 1994 Wiley‐Liss, Inc.
Increased natural antibody activity in sera of patients with malignant non‐Hodgkin's lymphomas containing paraproteins
Authors: Kourtis Athena P. , Boussiotis Vassiliki A. , Lymberi Pigi , Pangalis Gerassimos A. 
Published: February 2007

AbstractIn view of the frequent association between malignant lymphoproliferative diseases and autoimmunity, we studied by ELISA the presence, lsotype, and specificity of natural antibodies in sera from 60 patients with malignant non‐Hodgkin's lymphomas and 70 healthy controls. The antigens selected were actin, tubulin, myosin, troponin, tropomyosin, keratin, thyroglobulin, single stranded DNA, and the hapten trinitrophenyl. The results of this study were compared with the presence of oligoclonal immunoglobulins in the same sera, as previously detected by lsoelectric Focusing and Immunoblotting.Our results showed that 1) there is a high proportion of autoreactive NHL sera: 28 (46.6%) exhibited high IgG natural antibody activity (Nab) against one or more panel antigens, mostly against keratin, myosin, and tropomyosin, 26 (43.3%) had high IgA Nab activity, mainly against myosin, keratin, and ssDNA, whereas 17 (28.3%) displayed a high IgM Nab activity. The great majority (>80%) of positive sera was polyspecific, i.e., reacted with at least two panel antigens. 2) A strong correlation exists between the high serum Nab activity and the presence of paraproteins: of the 28 sera exhibiting high IgG Nab activity, 16 (57%) contained oligoclonal IgG, and of the 17 sera exhibiting high IgM Nab activity, 13 (76.4%) contained oligoclonal IgM. The Nabs detected had frequently the same heavy and light chain as the paraproteins. No relationship between the presence of paraproteins with Nab activity and the stage of disease or grade of malignancy was found. We conclude that the high incidence of clonal immunoglobulins of different isotypes with various autoantibody specifities suggests an oligoclonal lymphocyte activation involving Nab producing cells. The significance of such autoreactive clones in NHL patients remains to be elucidated. © 1994 Wiley‐Liss, Inc.
Low‐dose ARA‐C consistently induces hematologic responses in the clinical 5q–syndrome
Authors: Juneja Harinder S. , Jodhani Madhu , Gardner Frank H. , Trevarthen David , Schottstedt Margaret 
Published: February 2007

AbstractFive patients with the classical clinical syndrome associated with a deletion of the long arm of chromosome 5, i.e., anemia, macrocytosis, and thrombocytosis, or a normal platelet count, were treated successfully with subcutaneous low‐dose cytosine arabinoside (LDARA‐C). Prior therapy with other drugs had failed in four of the five patients. A total of nine complete and one partial hematologic responses were induced in five patients. Duration of the first hematologic response ranged from 3 to 30+ months. Two patients (cases 3 and 4) continue in their first hematologic response at 29 and 30 months. Upon relapse, up to three responses could be reinduced in two patients. Duration of the subsequent hematologic responses in case 1 was 16, 8, and 10 months and case 2 achieved two responses of 15 and 18+ months duration. LDARA‐C therapy was associated with mild to severe neutropenia and moderate to severe thrombocytopenia. Thus, subcutaneous LDARA‐C is highly effective in the treatment of patients with myelodysplasia associated with deletion of the long– arm of chromosome 5 (5q–). © 1994 Wiley‐Liss, Inc.
Molecular analysis of an asymptomatic Ph‐positive CML patient with 27 years of prolonged remission
Authors: Amikam D. , Henig C. , Sharon R. , Tatarsky I. , Ben‐Ishai Z. 
Published: February 2007

AbstractWe describe the results of clinical, cytogenetic, and molecular biological studies of a patient who, after two courses of treatment with Busulphan, has remained free of symptoms of Philadelphia chromosome‐positive chronic myeloid leukemia for 27 years, except for the persistence of a Ph1 chromosome and the presence of its transcribed chimeric mRNA. © 1994 Wiley‐Liss, Inc.
Inhibition by aurintricarboxylic acid of von Willebrand factor binding to platelet GPIb, platelet retention, and thrombus formation in vivo
Authors: Kawasaki T. , Kaku S. , Kohinata T. , Sakai Y. , Taniuchi Y. , Kawamura K. , Yano S. , Takenaka T. , Fujimura Y. 
Published: February 2007

AbstractCommercial aurintricarboxylic acid (ATA) was separated into molecular‐weight (MW) fractions of <210 to >25,000, using gel permeation chromatography. Fractions with MW >1,300 effectively inhibited both botrocetin‐induced vWF and bovine vWF binding to fixed human platelets. These activities decreased with a MW >17,000. Platelet retention for a human in vitro was reduced by ATA at 150 μ, as was that for rats ex vivo at 3 mg/kg. ATA prolonged tail transection bleeding time in rats but had only a weak effect on buccal mucosal bleeding time in dogs. ATA had no effect on platelet count but markedly prolonged PTT. ATA at 10 mg/kg exhibited antithrombotic activity and caused a marked improvement in patency status following successful thrombolysis by t‐PA in electrically and copper coil‐induced thrombosis models. These results suggest that specific inhibitors of the vWF‐GPIb interaction such as ATA may prove useful as antithrombotic agents. © 1994 Wiley‐Liss, Inc.
Langerhans cell histiocytosis: Lack of a viral etiology
Authors: McClain Kenneth , Jin Hong , Gresik Vicky , Favara Blaise 
Published: February 2007

AbstractLangerhans cell histiocytosis (LCH) is an enigmatic histiocytic proliferative disorder of unknown etiology that affects children primarily. We have investigated the possibility that viruses are etiological or that they have a “triggering effect” in LCH. Sensitive in situ hybridization and polymerase chain reaction (PCR) techniques were used in 56 cases of LCH. We sought and failed to find evidence of genomes for adenovirus, cytomegalovirus, Epstein‐Barr virus, herpes simplex virus, human herpesvirus type 6, human immunodeficiency virus, human T‐cell leukemia virus types I and II, and parvovfrus. Although some probes hybridized to tissues from several cases, PCR failed to confirm the presence of viral genome in any. We conclude that there is no evidence that these viruses are associated with LCH. © 1994 Wiley‐Liss, Inc.
DNA diagnosis of Hb S and Hb Caribbean (α2β291 Leu→Arg) in a Jamaican family
Authors: Waye John S. , Patterson Margaret , Eng Barry , Chui David H. K. , Sher Graham D. , Olivieri Nancy F. 
Published: February 2007

AbstractWe describe a Canadian infant of Jamaican descent who presented with mild anemia. Hb electrophoresis revealed Hb S and an unknown Hb variant that migrated slightly faster than Hb S on cellulose acetate. Molecular studies of the family indicated that the proband is a compound heterozygote for Hb S and Hb Caribbean. Hb Caribbean has previously been characterized as a mildly unstable hemoglobin with low oxygen affinity, due to a Leu→Arg substitution at amino acid residue 91. The present study establishes the molecular basis for Hb Caribbean (β91, CTG→CGG) and confirms that Hb S/Hb Caribbean syndrome is not associated with serious clinical manifestations. © 1994 Wiley‐Liss, Inc.
Polyethylene glycolated interleukin‐2 as maintenance therapy for acute myelogenous leukemia in second remission
Authors: Wiernik Peter H. , Dutcher Janice P. , Todd Mary , Caliendo Geralyn , Benson Laura 
Published: February 2007

AbstractSeven patients in second complete remission of acute myeloid leukemia (AML) aged 19–65 years were treated with polyethylene glycolated interleukin‐2 (PEG IL‐2), 1 × 106 U/M2 IV weekly as the sole postremission therapy. Second remission duration was in the range of 4–49+ months, and three patients had a second remission duration substantially longer than their first (6,21 +, and 42+ months). The results suggest that PEG IL‐2 may prolong second remission duration in AML and that a prospective randomized study designed to test that idea is warranted. © 1994 Wiley‐Liss, Inc.
Bone marrow necrosis in two patients with acute promyelocytic leukemia during treatment with all‐trans retinoic acid
Authors: Limentani Steven A. , Pretell Judith O. , Potter David , Dubois Jon S. , Daoust Philip R. , Spieler Paul S. , Miller Kenneth B. 
Published: February 2007

AbstractAll‐trans retinoic acid has been used for the treatment of acute promyelocytic leukemia (APL) with encouraging results. However, it has recently been associated with a number of potentially serious complications including the retinoic acid syndrome. We describe two patients with APL who were begun on all‐trans retinoic acid therapy (45 mg/m2), but who developed leukocytosis which was treated with hydroxyurea. Both patients demonstrated clinical and laboratory findings of disseminated intravascular coagulation, massive cell lysis manifested by marked increases in serum lactic dehydrogenase, and rapid clinical deterioration. Both patients developed bone marrow necrosis within viable, non‐infarcted bone trabeculae. We postulate that the development of bone marrow necrosis in these two patients was not a chance occurrence. Rather, the specific combination of cytotoxic and differentiating agents used in these patients (hydroxyurea with all‐trans retinoic acid) caused massive cell lysis and death. The absence of bone marrow necrosis in the setting of induction therapy for APL both with and without all‐trans retinoic acid therapy suggests that the addition of hydroxyurea was critical to the development of marrow necrosis. We, therefore, recommend caution in the use of hydroxyurea and all‐trans retinoic acid in the treatment of APL. © 1994 Wiley‐Liss, Inc.
IL‐5 mRNA expression in blood lymphocytes from patients with Kimura's disease and parasite infection
Authors: Enokihara Hideo , Koike Toshiyuki , Arimura Hironari , Aoyagi Masakuni , Watanabe Kanji , Nakamura Yuichi , Yamashiro Kyoko , Tsuruoka Nobuo , Tsujimoto Masafumi , Saito Kenji , Furusawa Shimpei , Shishido Hideo 
Published: February 2007

AbstractBlood lymphocytes from patients with eosinophilia are known to produce interleukin‐5 (IL‐5) with appropriate stimulation in vitro. To determine whether blood lymphocytes from these patients produce IL‐5 in vivo, we tested the IL‐5 mRNA expression in blood lymphocytes immediately after separation by reverse‐transcriptase polymerase chain reaction (RT‐PCR) method. We found that lymphocytes from eosinophilic patients expressed IL‐5 mRNA, but lymphocytes from normal volunteers did not express the lymphokine. These findings suggest that in patients with eosinophilia, peripheral blood lymphocytes produce IL‐5 in vivo. © 1994 Wiley‐Liss, Inc.
Improvement of sickle cell anemia by iron‐limited erythropoiesis
Authors: Castro Oswaldo , Poillon William N. , Finke Helga , Massac Etienne 
Published: February 2007

AbstractWe report the hematologic and clinical features of four adult patients (Pts.) with sickle cell anemia and iron‐limited erythropoiesis. Two of the Pts. had spontaneous iron deficiency (chronic Gl bleeding, low‐grade hemoglobinuria). In the other two Pts. iron restriction was induced by periodic RBC aphereses as part of a pilot protocol designed to decrease intracellular HbS polymerization by MCHC reduction. Iron‐limited erythropoiesis was defined by reduction in red cell indices (MCV range 60.4–67 fl) in the presence of low serum ferritin (range <10–20 ng/ml). In these Pts. iron restriction did not cause clinically significant worsening of the anemia (Hb 7.8–9.0 g/dl). In two Pts. the anemia actually improved. Other hematologic effects of iron restriction were: decreased MCHC, reticulocyte count, RDW, and dense cells. A reduced hemolytic rate was suggested by a lowering of serum bilirubin and LDH. In one of the Pts. the 51Cr RBC T1/2 survival increased from 12 to 16 days. The intracellular HbS polymer fractions (fp) were determined at 25% O2 by Csat and with the use of the conservation of mass equation. The baseline fp values ranged from 0.48–0.53. After iron restriction they ranged from 0.33–0.48. The fp decreased even though iron‐limited erythropoiesis also lowered the Hb F concentration in three of our Pts. In one of the two Pts. with induced iron depletion, hospitalization days for pain crises decreased from an average of 4.5 days/month (2 year baseline period) to an average of 0.5 days/month in the 3 year follow‐up after iron depletion. The second patient with induced iron restriction experienced the rapid healing of a leg ulcer. Controlled iron restriction should be explored as a therapeutic strategy in selected SS patients. © 1994 Wiley‐Liss, Inc.
Plasma levels of granulocyte elastase‐α1‐proteinase inhibitor complex in patients with disseminated intravascular coagulation: Pathophysiologic implications
Authors: Okajima Kenji , Fujise Ryuji , Motosato Yuichi , Ushijima Masato , Okabe Hiroaki , Takatsuki Kiyoshi 
Published: February 2007

AbstractTo Investigate the role of neutrophil activation in the pathophysiology and sequelae of disseminated intravascular coagulation (DIC), we measured plasma levels of granulocyte elastase‐α1‐proteinase inhibitor complex (GEPIC) in 41 patients with DIC and 27 patients with similar underlying conditions but without DIC. Mean GEPIC levels were significantly higher in patients with DIC (421.0 ± 45.6 ng/ml) than in patients without DIC (246.1 ± 41.9 ng/ml, P < 0.01). Significant differences were also noted in DIC patients with or without infection (474.7 ± 61.2 ng/ml vs. 302.4 ± 48.9 ng/ml, P < 0.04), with or without organ dysfunction (546.6 ± 72.7 ng/ml vs. 305.6 ± 42 ng/ml, P < 0.01), and with or without respiratory failure (640.0 ± 91.2 ng/ml vs. 328.1 ± 55.1 ng/ml, P < 0.01). No significant difference was found in mean GEPIC levels in DIC patients with or without renal failure, heart failure, hepatic failure, or gastrointestinal bleeding. The frequency of respiratory failure correlated with rising plasma levels of GEPIC. Mortality was higher in patients with GEPIC levels >500 ng/ml (53.8%) than in patients with GEPIC levels <500 ng/ml (28.6%). This correlation was particularly strong in patients with DIC, infection, and respiratory failure. Based on these data, we suggest that neutrophil activation, triggered by the coagulation cascade and perhaps augmented by endotoxin or cytokine release with infection, significantly contributes to respiratory failure and mortality in patients with DIC. © 1994 Wiley‐Liss, Inc.