Abstract:
:Two unrelated patients with Bernard-Soulier syndrome and their relatives were studied. The patients demonstrated severe bleeding diathesis, the relatives were asymptomatic. The propositi showed the characteristic abnormalities of the syndrome: thrombocytopenia, a percentage of giant platelets higher than 65%, prolonged bleeding time and defective platelet aggregation to ristocetin and bovine plasma. On the contrary, in the heterozygotes, the typical abnormalities were not fully evident. We introduce a simple ELISA method for the precise definition of both homozygous and heterozygous states for the syndrome by the quantitation of platelet glycoprotein (GP) Ib. Specific binding of monoclonal antibodies anti-platelet GPIb was performed both by direct binding of radioiodinated antibody and by ELISA. Comparable results were obtained. In fact, we demonstrated near absence of GPIb in the 2 propositi and about half the amount in the heterozygotes studied compared to normal platelets.
journal_name
Acta Haematoljournal_title
Acta haematologicaauthors
De Marco L,Fabris F,Casonato A,Fabris P,Dal Ben MG,Barbato A,Girolami Adoi
10.1159/000206125subject
Has Abstractpub_date
1986-01-01 00:00:00pages
203-8issue
4eissn
0001-5792issn
1421-9662journal_volume
75pub_type
杂志文章abstract::Treating patients with hemophilia and inhibitors is often problematic. The presence of inhibitors negatively impacts the effectiveness of treatment to achieve hemostasis especially in patients with hemophilia B, owing mainly to allergic reactions to factor IX (FIX) concentrates and the low success rate of immune toler...
journal_title:Acta haematologica
pub_type:
doi:10.1159/000508722
更新日期:2020-07-22 00:00:00
abstract::Leukemic cells of a patient with acute T-lymphoblastic leukemia (T-ALL) exhibiting the uncommon clinical feature of hypogammaglobulinemia were examined in terms of surface markers and immunologic functions. Employing various monoclonal reagents reacting with surface antigens present on T cells and additional conventio...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206496
更新日期:1984-01-01 00:00:00
abstract::A patient with regional enteritis had received iron dextran for treatment of iron deficiency. Subsequently he developed a large (3.1 g/100 ml) IgG-K serum spike which had precipitin activity against dextran sulfate but not a variety of other antigens. There has been no evidence of multiple myeloma and the spike gradua...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207755
更新日期:1978-01-01 00:00:00
abstract::Haemoglobin synthesis was studied in bone marrow erythroblasts and in reticulocytes of 4 children with beta O-thalassaemia major and of 7 children with beta +-thalassaemia major. In patients with beta O-thalassaemia the gamma/a ratio was found to be lower in bone marrow than in peripheral blood. On the contrary, in pa...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207174
更新日期:1981-01-01 00:00:00
abstract::The level of granulocyte-macrophage colony-stimulating factor (GM-CSF) in the cerebrospinal fluid from 14 infants and children with meningitis and 6 patients who suffered other diseases besides meningitis was measured by our sensitive enzyme linked immunosorbent assay for GM-CSF. The minimal detection level of GM-CSF ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204796
更新日期:1991-01-01 00:00:00
abstract::Iron deficiency is the main cause of anemia in both sexes, with women being more commonly affected. Iron therapy is currently considered an effective and safe remedy to replenish the iron storages. Iron can be administrated both orally and intravenously. In particular, intravenous (IV) iron therapy is widely used when...
journal_title:Acta haematologica
pub_type:
doi:10.1159/000503768
更新日期:2020-01-01 00:00:00
abstract::Hypersensitivity vasculitis occurs in response to various exogenous agents. A case of a serum sickness syndrome with cutaneous vasculitis is described in a patient given an intravenous iron-dextran infusion. ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205198
更新日期:1990-01-01 00:00:00
abstract::Successive chromatographic procedures made it possible to isolate thrombocytopoietin from the serum of thrombocytopenic rats. The following steps were taken: DEAE-cellulose phosphate chromatography, Sephadex chromatography, exclusion chromatography on DEAE-Sephadex A-50 gel. The apparent molecular weight of thrombocyt...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206900
更新日期:1983-01-01 00:00:00
abstract::The first thrombocytopenia cases related to the human immunodeficiency virus (HIV) were described even before its isolation in 1983. Subsequently, multiple mechanisms have been proposed to elucidate the etiology of thrombocytopenia. In addition to other types of cytopenia affecting patients with HIV, thrombocytopenia ...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000313782
更新日期:2010-01-01 00:00:00
abstract::We describe a patient with hemophagocytic syndrome resembling malignant histiocytosis which was complicating myelodysplastic disease of 3 years duration. Detailed morphological and ultrastructural studies indicate that the histiocytic component did not demonstrate features of malignancy. A review of other known malign...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206198
更新日期:1985-01-01 00:00:00
abstract:OBJECTIVE:To review the diagnostic significance, safety and possible risk factors of splenectomy in fever of unknown origin (FUO) with splenomegaly. METHODS:The records of 54 patients with FUO and splenomegaly who underwent splenectomy in our hospital in the past 20 years were reviewed retrospectively. Pathologic find...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000118632
更新日期:2008-01-01 00:00:00
abstract::The establishment of mouse and human monoclonal anti-DNA antibodies is described. Common idiotypes were identified on these antibodies employing monoclonal (mouse) and polyclonal anti-idiotypic antibodies. The significance of the presence and titer of the common idiotypes as clinical activity marker was examined in pa...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206029
更新日期:1986-01-01 00:00:00
abstract::A mild anemia (hemoglobin 9 g/dl) was found in a patient from Seville (Spain) with marked morphological abnormalities in the peripheral blood smear. The red cell osmotic fragility showed a mild resistance curve with a mean cell fragility (MCF) of 0.375% NaCl (normal = 0.450). Chemical Chemical and thermal instability ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206274
更新日期:1985-01-01 00:00:00
abstract::An ultrastructural study of the aspirated bone marrow of a patient with congenital dyserythropoietic anemia type I is presented. Both in transmission electron microscopy (TEM) and in scanning electron microscopy (SEM) ultrastructural abnormalities of the bone marrow erythroid precursors were seen. These abnormalities ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206735
更新日期:1983-01-01 00:00:00
abstract::We report on a patient with chronic myeloid leukemia (CML) with a rapidly growing left cervical tumor 5 months after the initial diagnosis of CML. This tumor was diagnosed as a very early manifestation of extramedullary myeloblastoma by a minimally invasive method. A fine needle aspirate (26-gauge needle) was obtained...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000040873
更新日期:1998-01-01 00:00:00
abstract::We examined the in vivo effect of granulocyte colony-stimulating factor (G-CSF) on the surface expression of putative counterligands for endothelial selectins on neutrophils in healthy volunteers. G-CSF (50 microg/m2/day) was administered subcutaneously to 5 healthy volunteers for 4 days. The expression of surface ant...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000040901
更新日期:1998-01-01 00:00:00
abstract::95 of 1,019 (9.3%) sera with monoclonal immunoglobulins (MIg) were found to have cold-reacting lymphocytotoxins (LCT). There was no difference in the prevalence of LCT in multiple myeloma, macroglobulinemia, cancer, lymphoma or benign monoclonal gammopathy. Prevalence of LCT was similar in various classes and types of...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206328
更新日期:1985-01-01 00:00:00
abstract::Recently, a factor was discovered in the serum of hepatectomized animals which was capable of augmenting the hepatic erythropoietin response to hypoxia when injected into normal rats. This substance was localized in the liver via an in situ perfusion technique and was termed the hepatic erythropoietic factor (HEF). Pa...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206884
更新日期:1983-01-01 00:00:00
abstract::The activity of ten red blood cell enzymes, including hexokinase, has been measured in 6 Fanconi's anemia patients. In disagreement with previous reports, in no instance were reduced or increased hexokinase levels found. Furthermore, the hexokinase isozymic pattern, thermostability, pH dependence of activity and kinet...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206612
更新日期:1984-01-01 00:00:00
abstract::Two human stromal cell lines were established previously from bone marrow-derived primary long-term cultures by immortalization using the SV40 large T antigen and cellular cloning. After irradiation, the fibroblast-like cell lines L87/4 and L88/5 support hematopoietic differentiation of allogeneic cord blood cells in ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000046559
更新日期:2001-01-01 00:00:00
abstract::Automated cytochemistry by the Hemalog D carries out leukocyte differential counts in a continuous flow mode on whole blood EDTA-collected samples, through the optical measurement of enzyme activity and cell size. This operative principle thus parallels the basis of the FAB classification of acute leukemias. Although ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206394
更新日期:1984-01-01 00:00:00
abstract::Slow intravenous infusion of Na laurate (NaL) into guinea pigs caused a rapid appearance of platelet aggregates in the arterial blood and a precipitous fall in platelet counts. During the infusion of Na arachidonate (NaA) thrombocytopenia developed slowly, and few and smaller platelet aggregates appeared in the arteri...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207864
更新日期:1977-01-01 00:00:00
abstract::Avascular necrosis (AVN) of the hip is a common cause of morbidity in sickle cell disease (SCD). Its prevalence increases with age and predisposing factors include coexistent alpha-thalassemia trait, frequent vaso-occlusive crisis and a high hematocrit (Hct). SCD is relatively mild among Kuwaiti patients because of th...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000046529
更新日期:2001-01-01 00:00:00
abstract:BACKGROUND:Our aim is to determine comorbidities associated with pulmonary hypertension (PHT) in clinically stable sickle-cell disease (SCD) patients and to evaluate left ventricular (LV) and right ventricular (RV) function in those patients. METHODS:Echocardiography was performed in 87 SCD patients that were divided ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000102588
更新日期:2007-01-01 00:00:00
abstract::Alpha-thalassemia is very common in the Kuwaiti population, but its influence on anemia of pregnancy has not been previously investigated. We have screened a group of 59 anemic (Hb < 11 g/dl) pregnant women for the alpha-thal-2 (-alpha-3.7 kb) deletion which is the commonest alpha-thal allele in this community, using ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000039757
更新日期:2000-01-01 00:00:00
abstract::A 56-year-old male presented with inguinal lymphadenopathy and leucocytosis (WBC 98 x 10(9)/l). Bone marrow morphology showed myeloid hyperplasia, with eosinophilia. Cytogenetic analysis showed no evidence of the Philadelphia chromosome, and fluorescence in situ hybridisation studies for the BCR/ABL fusion were negati...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000046638
更新日期:2002-01-01 00:00:00
abstract::Eltrombopag is a thrombopoietin receptor agonist frequently used to manage immune thrombocytopenia and aplastic anemia. At the high doses used for aplastic anemia, but not the doses used for immune thrombocytopenia, eltrombopag can cause reddish-brown discoloration of plasma, which can interfere with bilirubin measure...
journal_title:Acta haematologica
pub_type:
doi:10.1159/000508599
更新日期:2020-07-22 00:00:00
abstract::The transcription factors GATA-1 and GATA-2 play key roles in gene regulation during erythropoiesis. Gene ablation studies in mouse revealed that GATA-2 is crucial for the maintenance and proliferation of immature hematopoietic progenitors, whereas GATA-1 is essential for the survival of erythroid progenitors as well ...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000065660
更新日期:2002-01-01 00:00:00
abstract::A new deletion of the beta-globin gene cluster was characterized in a Turkish family. A 6-year-old male and his father were heterozygotes for this deletion. They presented with mild hypochromic microcytic anemia associated with elevated Hb F (15%) and normal Hb A2 levels (2.0%). This newly described Turkish type (delt...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000203790
更新日期:1996-01-01 00:00:00
abstract::2-Chlorodeoxyadenosine (cladribine, Leustatin) is being used extensively in the treatment of hematologic malignancies, but relatively little is known regarding its toxicity to the normal marrow. Long-term serial hematologic observations have been made on 29 patients with multiple sclerosis undergoing experimental ther...
journal_title:Acta haematologica
pub_type: 临床试验,杂志文章
doi:10.1159/000204236
更新日期:1994-01-01 00:00:00