Abstract:
:von Willebrand disease (VWD) type 2M is a distinct entity and clearly differs from type 1. The genotype-phenotype correlation for cases with ristocetin cofactor activity (RCo)/antigen (Ag) ratios <0.60 is clear, whereas the von Willebrand factor (VWF):collagen binding (CB)/VWF:Ag ratio is normal in VWD 2M. Typical laboratory features of VWD type 2 M are decreased ristocetin-induced platelet aggregation in the presence of a normal or near normal VWF multimeric pattern on a low-resolution agarose gel, a poor response to desmopressin (DDAVP) of VWF:RCo, and a good response of both VWF:CB and VWF:Ag to DDAVP. The phenotypic definition of VWD type 2M may need to be more stringent and should be the subject of an international standardization initiative.
journal_name
Acta Haematoljournal_title
Acta haematologicaauthors
Hermans C,Batlle Jdoi
10.1159/000214854subject
Has Abstractpub_date
2009-01-01 00:00:00pages
139-44issue
2-3eissn
0001-5792issn
1421-9662pii
000214854journal_volume
121pub_type
杂志文章,评审abstract::A 59-year-old man with beta-thalassaemia major is unusually well. He has no beta-chains in his haemoglobin but is heterozygous for the genes responsible for alphaA and for alphaG Philadelphia. In addition he is also heterozygous for the genes responsible for gammaF and a new gamma-chain, gamma75(E19) Ile-Thr, named ga...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000208204
更新日期:1975-01-01 00:00:00
abstract::Fetal liver transplantation was attempted in 7 patients with aplastic anemia. 4 of these patients showed a partial response as evidenced by decrease in blood transfusion requirements and increase in the peripheral blood counts and hematopoietic cells in the bone marrow. Bone marrow culture studies revealed evidence of...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207558
更新日期:1979-01-01 00:00:00
abstract::In pregnant mice. 55Fe-labeled iron-dextran (Imferon) is transferred across the placenta. It was detected in the bone marrow, liver, spleen and peripheral blood of the pregnant animal, as well as in the embryonic liver erythroid precursors and peripheral blood. Uptake by liver and peripheral blood cells of pregnant an...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000208195
更新日期:1975-01-01 00:00:00
abstract::The presence of the sickle cell (Hb S) gene in Saudi Arabia was first reported by Lehmann et al. in 1963 [Nature 198, pp. 492-493]. Later, Hb S, alpha- and beta-thalassaemia, glucose-6-phosphate dehydrogenase deficiency and other enzymopathies were shown to occur at a variable prevalence in different regions of the co...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205861
更新日期:1987-01-01 00:00:00
abstract::Agranulocytosis developed in a 20-year-old Greek patient with beta-thalassaemia major, 11 weeks after commencing chelation with the oral iron chelator 1,2-dimethyl-3-hydroxypyrid-4-one (L1) and 6 weeks after receiving the drug at a total daily dose of 105 mg/kg. The patient presented with generalised weakness, low-gra...
journal_title:Acta haematologica
pub_type: 临床试验,杂志文章
doi:10.1159/000204494
更新日期:1993-01-01 00:00:00
abstract::A new variant of red cell glucose-6-phosphate dehydrogenase (G6PD) has been found in a Caucasian man with congenital non-spherocytic haemolytic anaemia. This variant has reduced activity, increased thermolability, increased Michaelis constants for glucose-6-phosphate and NADP, slightly increased electrophoretic mobili...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207869
更新日期:1977-01-01 00:00:00
abstract:BACKGROUND:The relation with SNF5 mutation and chromosome 22 abnormalities is not clear in hematological neoplasms. METHODS:To elucidate the relevance of the SNF5 gene on 22q11.2, karyotypes were reviewed in 283 hematological neoplasms. Loss of heterozygosity (LOH) on 22q was analyzed in 21 plasma cell myelomas withou...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000324932
更新日期:2011-01-01 00:00:00
abstract::Macrothrombocytopenia (MTP) is a group of rare disorders characterized by giant platelets, thrombocytopenia, and variable association with abnormal bleeding. Inherited MTP are frequently misdiagnosed as immune thrombocytopenia. Associated second-organ manifestation can help narrow down syndromic MTPs. We describe a ca...
journal_title:Acta haematologica
pub_type:
doi:10.1159/000506727
更新日期:2021-01-01 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:BACKGROUND:Immunotherapy using recombinant human interleukin-2 (rIL-2) produces objective responses in a proportion of advanced cancer patients. While early investigators employed intravenous (i.v.) treatment regimens, recent clinical trials applied therapy schedules via subcutaneous (s.c.) injection, mostly in combina...
journal_title:Acta haematologica
pub_type: 临床试验,杂志文章
doi:10.1159/000204503
更新日期:1993-01-01 00:00:00
abstract::A 27-year-old male developed massive generalized lymphadenopathy with chronic myelomonocytic leukemia (CMML) presenting as extramedullary blast crisis mimicking a lymphocytic lymphoma. On presentation, a consistent chromosomal abnormality involving chromosomes 8 and 13, i.e. 46,XY,t(8;13) (q11;p11), was present in lym...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204687
更新日期:1992-01-01 00:00:00
abstract:BACKGROUND:Danaparoid sodium and synthetic protease inhibitors (SPIs) have been approved for the treatment of disseminated intravascular coagulation (DIC) in Japan. OBJECTIVES:To compare the clinical results of the treatment of DIC with danaparoid or SPIs. METHODS:We retrospectively examined 188 patients with hematol...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000501818
更新日期:2020-01-01 00:00:00
abstract::An unusual complication of chronic lymphocytic leukemia (CLL) is reported. The patient, a 79-year-old man, had a long standing history of CLL, that had been complicated by the development of a Guillain-Barré-like syndrome and a peripheral biclonal gammopathy. The biclonal immunoglobulins identified in the serum were I...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204593
更新日期:1992-01-01 00:00:00
abstract::Dihydrofolate reductase (FH2-R) was studied cytochemically in the bone marrow erythroblasts of 20 normal controls and 46 patients with myelodysplastic syndromes (MDSs) classified according to FAB, prior to therapy. The reaction product was quantified for the same samples with a Vickers M86 microdensitometer. The enzym...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205807
更新日期:1988-01-01 00:00:00
abstract::A variety of adhesion receptors are expressed on the blast cells in patients with acute myelogenous leukemia. The panel of receptors expressed demonstrates heterogeneity just as there is morphologic, histochemical, cytogenetic, and molecular genetic variation between various cases of acute myelogenous leukemia. The ad...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000203659
更新日期:1997-01-01 00:00:00
abstract::The last few years have seen an enormous increase in our knowledge on the haematopoietic growth factor erythropoietin (Epo), firstly with its purification and determination of its primary amino acid sequence, and more recently with the isolation of the Epo gene and its expression in mammalian cell lines. This review a...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000204826
更新日期:1991-01-01 00:00:00
abstract::Platelet aggregation in citrated and heparinized plasma by ionophore A 23187 and Ristocetin was studied in normal subjects and in patients with von Willebrand's disease and congenital afibrinogenemia. Aggregation by ionophore was normal in all groups both in citrated and heparinized plasma. Aggregation by Ristocetin i...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207932
更新日期:1976-01-01 00:00:00
abstract::Residues 124 and 125 of the beta-chain of human haemoglobin are prolyl-prolyl. In the delta-chain, one of these positions is occupied by a glutaminyl, and there has been uncertainty as to which of the two residues is the prolyl and which the glutaminyl. The sequence has now been established to be delta124 Pro - 125 Gl...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207923
更新日期:1976-01-01 00:00:00
abstract::Platelet aggregation in diabetic retinopathy was investigated in a group of 25 patients. An enhanced activity induced by epinephrine and arachidonic acid was found in this group as compared with the controls, whereas in adenosine diphosphate (ADP) platelet aggregation no differences were observed. Spontaneous aggregat...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207695
更新日期:1978-01-01 00:00:00
abstract::Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is a rare lymphoproliferative disorder characterized by diffuse lymphadenopathy, fever, hepatosplenomegaly, hemolytic anemia, and polyclonal hypergammaglobulinemia. Morphologically, the involved lymph nodes demonstrate complete effacement of the normal arch...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000040811
更新日期:1998-01-01 00:00:00
abstract::We evaluated the results of high-dose therapy (HDT) and autologous hematopoietic stem cell transplantation (ASCT) in patients with relapsed or primary refractory Hodgkin's disease (HD), using a previously reported prognostic model based on the presence of three poor prognostic factors at the start of salvage therapy/p...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000074220
更新日期:2003-01-01 00:00:00
abstract::Deficiency in glucose-6-phosphate dehydrogenase (G6PD) is the most common enzymopathy, and more than 125 different mutations causing G6PD deficiency have been identified. Chronic haemolytic anaemia (CHA) associated with G6PD deficiency is rare, but there is a cluster of mutations causing CHA between amino acids 361-42...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000064749
更新日期:2002-01-01 00:00:00
abstract::To assess the effect of prophylactic treatment with antithymocyte globulin (ATG) on graft-versus-host disease (GvHD) in myeloablative transplant patients, we performed a meta-analysis of randomized and cohort studies. Medline, Embase, the Cochrane Controlled Trial Register and the Science Citation Index were searched ...
journal_title:Acta haematologica
pub_type: 杂志文章,meta分析,评审
doi:10.1159/000343604
更新日期:2013-01-01 00:00:00
abstract::The most common single genetic disorder and a major public health issue in Greece and other Mediterranean countries is beta-thalassemia. Current therapeutic approaches for homozygous beta-thalassemia entail blood transfusions and iron chelation therapy with deferoxamine or deferiprone for preventing tissue hemosideros...
journal_title:Acta haematologica
pub_type: 临床试验,杂志文章
doi:10.1159/000077551
更新日期:2004-01-01 00:00:00
abstract:BACKGROUND:It is a common belief among women that iron compounds have unpleasant gastrointestinal side effects. OBJECTIVE:To assess the gastrointestinal side effects of iron prophylaxis in pregnancy. METHODS:A randomized, double-blind study comprising 404 healthy pregnant women allocated to four groups taking ferrous...
journal_title:Acta haematologica
pub_type: 杂志文章,随机对照试验
doi:10.1159/000089466
更新日期:2006-01-01 00:00:00
abstract::A case of steroid-resistant acquired pure red-cell aplasia associated with a relative and absolute increase of TG cells is described. Removal of T cells enhanced erythroid colony and burst formation in vitro, and it is suggested that TG cells may be implicated in the pathogenesis of this case of acquired pure red-cell...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207191
更新日期:1981-01-01 00:00:00
abstract::Malaria parasites growing inside human erythrocytes differ from mammalian cells in their mode of acquisition of bioavailable iron and in their susceptibility to the antiproliferative action of iron chelators. We have assessed here three major properties associated with these phenomena: (a) the stage-dependent nature o...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000203952
更新日期:1996-01-01 00:00:00
abstract::A rare case of acute promyelcytic leukemia (APL) is reported in a 7-year-old boy. The patient displayed the typical features of APL including impaction of the marrow with promyelocytes, marked elevation of the serum vitamin B12 and transcobalamin I levels and a hemorrhagic diathesis. The bleeding diathesis in the case...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207589
更新日期:1979-01-01 00:00:00
abstract::Glucocorticosteroid hormones have been reported either to stimulate or to inhibit human erythropoiesis. We have studied the in vitro effect of hydrocortisone, 10(-6) mol/l, on human BFU-E when stimulated by preconstituted burst-promoting activity (BPA) in a medium conditioned by T lymphocytes. Hydrocortisone was found...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205272
更新日期:1989-01-01 00:00:00
abstract::A 44-year-old Japanese male having refractory anemia with excess of blasts (RAEB) preceding acute myelomonocytic leukemia (AMMoL) with dysplastic marrow eosinophilia (M4Eo in the FAB classification) is reported. Sequential cytogenetic studies revealed a specific chromosomal abnormality, inv(16) (p13q22), when RAEB was...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205368
更新日期:1989-01-01 00:00:00