Abstract:
BACKGROUND:Splanchnic vein thrombosis (SVT) is a severe complication of essential thrombocythemia (ET). No clear explanation has been given for the occurrence of thrombosis in this unusual site in patients with ET, but the existence of a specific association between unexplained SVT and the JAK2 mutation has been reported. METHODS AND RESULTS:The present study describes SVT (portal and splenic vein thrombosis) in a young woman as the first presenting symptom of latent ET. Extensive screening for thrombophilia was negative. Our patient in fact did not fulfill the WHO diagnostic criteria for myeloproliferative disease (MPD), while she had splenomegaly and developed features suggestive of latent ET during follow-up. CONCLUSIONS:In these patients with SVT, the detection of JAK2(V617F) mutation is diagnostic for masked MPD as could be documented by bone marrow histopathology. The presence of JAK2(V617F) mutation should be considered per se a prothrombotic state for cerebral, coronary and peripheral microvascular disturbances and for SVT but not for deep vein thrombosis. Anticoagulation is the treatment of choice for all SVT and proper treatment of the MPD is recommended in patients with SVT associated with the JAK2(V617F) mutation.
journal_name
Acta Haematoljournal_title
Acta haematologicaauthors
Allegra A,Alonci A,Penna G,D'Angelo A,Rizzotti P,Granata A,Musolino Cdoi
10.1159/000221962subject
Has Abstractpub_date
2009-01-01 00:00:00pages
218-20issue
4eissn
0001-5792issn
1421-9662pii
000221962journal_volume
121pub_type
杂志文章abstract::In a dose titration study we tested the efficacy and tolerance of recombinant human erythropoietin (rhEPO) in 10 patients with myelodysplasia (MDS) and 2 patients with idiopathic myelofibrosis. Patients with a haemoglobin level < 100 g/l were treated as out-patients for 12 weeks with daily doses ranging from 30 U/kg b...
journal_title:Acta haematologica
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journal_title:Acta haematologica
pub_type: 杂志文章,meta分析
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abstract::The BCR-ABL fusion gene represents the hallmark of chronic myelogenous leukemia (CML) and is derived from a translocation between chromosome 9 and 22. The majority of CML patients have a breakpoint in the major BCR region of the BCR gene giving rise to e13a2 or e14a2 BCR-ABL transcripts. Occasionally, other BCR breakp...
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doi:10.1159/000230037
更新日期:2009-01-01 00:00:00
abstract::In order to study the abnormal differentiation and proliferation of precursor T cells in homosexual men, a study was made of T-cell colony formation in 15 healthy homosexual men without anti-human immunodeficiency virus (HIV) antibody. The colony forming method used was the one-step monolayer technique using phytohema...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205374
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abstract::It was found that neutrophils in untreated uraemic patients as well as in subjects on regular dialysis treatment displayed higher activity of acid phosphatase, alkaline phosphatase and peroxidase. Spontaneous reduction of nitro blue tetrazolium (NBT) by granulocytes was also higher in both groups in comparison to cont...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207647
更新日期:1979-01-01 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
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abstract::Serum IgG, IgM and IgA were determined in 25 patients with homozygous beta thalassemia and 7 with the trait. The levels were increased in homozygous patients and increased further after splenectomy. Serum opsonic activity against Salmonella typhi and staphylococci was impaired in homozygous patients. Splenectomy cause...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206875
更新日期:1983-01-01 00:00:00
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journal_title:Acta haematologica
pub_type: 杂志文章,评审
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journal_title:Acta haematologica
pub_type: 杂志文章
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pub_type: 杂志文章
doi:10.1159/000208185
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journal_title:Acta haematologica
pub_type: 杂志文章
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abstract::The 8p11 myeloproliferative syndrome (EMS) is an aggressive neoplasm associated with chromosomal translocations involving the fibroblast growth factor receptor 1 (FGFR1) tyrosine kinase gene on chromosome 8p11-12. A new case of a 9-year-old boy with leukocytosis, eosinophilia, and general lymphadenopathy is reported i...
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abstract::34 patients with myocardial infarction were studied with daily measurements of beta-thromboglobulin (BTG) and 125I fibrinogen scanning in order to detect deep-venous thrombosis (DVT). Serial levels of BTG were unhelpful in the early detection of this condition, which occurred in 7 of the patients studied. 5 of the pat...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207238
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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
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journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207231
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abstract::Twenty-four infants and children suffering from glucose-6-phosphate dehydrogenase (G6PD) deficiency during hemolytic crisis were included in this study. Their ages ranged between 3 and 36 months with a median of 10 months. 22 were males and 2 were females. Fourteen out of them received a single bolus dose of desferrio...
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pub_type: 临床试验,杂志文章,随机对照试验
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journal_title:Acta haematologica
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journal_title:Acta haematologica
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更新日期:1996-01-01 00:00:00
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pub_type: 杂志文章
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更新日期:2003-01-01 00:00:00
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更新日期:1990-01-01 00:00:00
abstract::Low-risk patients with myelodysplastic syndromes (MDS) are inclined to long-term accumulation of iron in the organs due mostly to red blood cell transfusion and ineffective erythropoiesis. The effect of free toxic iron species in the liver and heart sites is well known, but recent knowledge assumes that oxidant-mediat...
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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
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更新日期:2000-01-01 00:00:00
abstract::Recombinant alpha-interferons are used as therapeutic agents in an increasing number of benign and malignant disorders. Long-term administration of recombinant alpha-interferon as a maintenance agent is associated with a small number of adverse side-effects which are responsible for patient intolerance of this drug. T...
journal_title:Acta haematologica
pub_type: 杂志文章
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abstract::Thrombopoietin levels in thrombocytopenic mice assayed by 75Se-se-lenomethionine incorporation into blood platelets reached a maximum 12 h after the induction of an acute, immune thrombocytopenia; that was more than twice the value in control mice. The implications of this finding are discussed with reference to the k...
journal_title:Acta haematologica
pub_type: 杂志文章
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更新日期:1975-01-01 00:00:00
abstract::A diester lipase activity is described in human red blood cells (RBC). Diester lipase activity acts as a membrane-bound enzyme and is assayed using intact RBC as the enzyme source. An emulsion of di-[3H]-oleoylglycerol (0.6 mM) serves as the substrate. The optimum pH for the reaction is 7.8 at 37 degrees C. Lipolytic ...
journal_title:Acta haematologica
pub_type: 杂志文章
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更新日期:1983-01-01 00:00:00
abstract::It has been proposed that iron overload may adversely affect liver disease outcome. The recent identification of 2 mutations in the HFE gene related to hereditary haemochromatosis (Cys282Tyr and His63Asp) provided an opportunity to test whether they are associated with hepatic iron accumulation and the activity and se...
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