Document Detail


The JAK2 46/1 haplotype in Budd-Chiari syndrome and portal vein thrombosis.
MedLine Citation:
PMID:  21364191     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The germline JAK2 46/1 haplotype has been associated with the development of JAK2(V617F)-positive as well as JAK2(V617F)-negative myeloproliferative neoplasms (MPNs). In this study we examined the role of the 46/1 haplotype in the etiology and clinical presentation of patients with splanchnic vein thrombosis (SVT), in which MPNs are the most prominent underlying etiological factor. The single-nucleotide polymorphism rs12343867, which tags 46/1, was genotyped in 199 SVT patients. The 46/1 haplotype was overrepresented in JAK2(V617F)-positive SVT patients compared with controls (P < .01). Prevalence of the 46/1 haplotype in JAK2(V617F)-negative SVT patients did not differ from prevalence in the controls. However, JAK2(V617F)-negative SVT patients with a proven MPN also exhibited an increased frequency of the 46/1 haplotype (P = .06). Interestingly, 46/1 was associated with increased erythropoiesis in JAK2(V617F)-negative SVT patients. We conclude that the 46/1 haplotype is associated with the development of JAK2(V617F)-positive SVT. In addition, our findings in JAK2(V617F)-negative SVT patients indicate an important role for the 46/1 haplotype in the etiology and diagnosis of SVT-related MPNs, independent of JAK2(V617F), that requires further exploration.
Authors:
Jasper H Smalberg; Edith Koehler; Sarwa Darwish Murad; Aurelie Plessier; Susana Seijo; Jonel Trebicka; Massimo Primignani; Moniek P M de Maat; Juan-Carlos Garcia-Pagan; Dominique C Valla; Harry L A Janssen; Frank W G Leebeek;
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2011-03-01
Journal Detail:
Title:  Blood     Volume:  117     ISSN:  1528-0020     ISO Abbreviation:  Blood     Publication Date:  2011 Apr 
Date Detail:
Created Date:  2011-04-15     Completed Date:  2011-06-21     Revised Date:  2011-07-15    
Medline Journal Info:
Nlm Unique ID:  7603509     Medline TA:  Blood     Country:  United States    
Other Details:
Languages:  eng     Pagination:  3968-73     Citation Subset:  AIM; IM    
Affiliation:
Department of Hematology, Erasmus University Medical Center, Rotterdam, The Netherlands.
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MeSH Terms
Descriptor/Qualifier:
Adult
Budd-Chiari Syndrome / epidemiology,  genetics*
Case-Control Studies
Factor V / genetics
Female
Genetic Predisposition to Disease / epidemiology
Haplotypes
Humans
Janus Kinase 2 / genetics*
Male
Middle Aged
Portal Vein*
Prothrombin / genetics
Risk Factors
Splanchnic Circulation*
Venous Thrombosis / epidemiology,  genetics*
Chemical
Reg. No./Substance:
0/factor V Leiden; 9001-24-5/Factor V; 9001-26-7/Prothrombin; EC 2.7.1.112/JAK2 protein, human; EC 2.7.10.1/Janus Kinase 2
Comments/Corrections
Comment In:
Blood. 2011 May 26;117(21):5777-8   [PMID:  21617012 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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