Document Detail


[Budd-Chiari syndrome and splanchnic vein thrombosis: masked myeloproliferative neoplasms and JAK2V617F].
MedLine Citation:
PMID:  20499034     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
The Budd-Chiari Syndrome (BCS) and the splanchnic vein thrombosis are characterized by hepatic venous outflow obstruction, generally due to venous thrombosis. These rare diseases are usually caused by multiple concurrent factors, including acquired and inherited thrombophilias. Since the diagnosis of myeloproliferative neoplasms (MPNs) is often difficult in patients with BCS and splanchnic vein thrombosis because of spleen enlargement, secondary pancytopenia and bleeding disorders, recent observations have included in the diagnostic work-up the analysis of the JAK2 mutation. The revision of several recent reports clarify the importance of the JAK2V617F detection in the diagnostic work-up of the BCS and splanchnic vein thrombosis, allowing the demonstration of masked MPNs among these cases that may benefit, in the near future, of target molecular therapies directed toward the JAK2 mutation.
Authors:
A Fama; A Rago; F Gioiosa; C Marzano; R Latagliata; C Mammì; C Laganà; G M D'Elia; L Bizzoni; S Trasarti; A Ferretti; M Breccia; O Riggio; A Tafuri
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Publication Detail:
Type:  English Abstract; Journal Article    
Journal Detail:
Title:  La Clinica terapeutica     Volume:  161     ISSN:  1972-6007     ISO Abbreviation:  Clin Ter     Publication Date:  2010  
Date Detail:
Created Date:  2010-05-25     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0372604     Medline TA:  Clin Ter     Country:  Italy    
Other Details:
Languages:  ita     Pagination:  169-71     Citation Subset:  IM    
Affiliation:
Dipartimento di Biotecnologie Cellulari ed Ematologia, Università Sapienza, Roma, Italia.
Vernacular Title:
Sindrome di Budd-Chiari e trombosi delle vene splancniche: neoplasie mieloproliferative mascherate e mutazione di JAK2V617F.
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