Document Detail

Hepatic vein thrombosis in paroxysmal nocturnal hemoglobinuria. A spectrum from asymptomatic occlusion of hepatic venules to fatal Budd-Chiari syndrome.
MedLine Citation:
PMID:  3609666     Owner:  NLM     Status:  MEDLINE    
In a series of 40 patients with Budd-Chiari syndrome, 5 (12%) were found to be afflicted with paroxysmal nocturnal hemoglobinuria. The clinico-pathological features in these 5 patients and in 26 well-documented previously reported cases could be ascribed to three groups of increasing severity: thrombosis limited to small-sized hepatic veins with no or transient ascites, partial thrombosis of large-sized hepatic veins with chronic ascites, and complete thrombosis of large-sized hepatic veins with a life-threatening course. These three groups did not differ with regard to sex, age, and duration and characteristics of paroxysmal nocturnal hemoglobinuria. In view of the relationship between prognosis and the extent of hepatic vein obstruction, we suggest that early therapy directed toward limiting the extension of thrombosis, or toward dissolving formed thrombi, should improve the prognosis of this severe complication of paroxysmal nocturnal hemoglobinuria.
D Valla; D Dhumeaux; G Babany; P Hillon; B Rueff; H Rochant; J P Benhamou
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Gastroenterology     Volume:  93     ISSN:  0016-5085     ISO Abbreviation:  Gastroenterology     Publication Date:  1987 Sep 
Date Detail:
Created Date:  1987-09-09     Completed Date:  1987-09-09     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0374630     Medline TA:  Gastroenterology     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  569-75     Citation Subset:  AIM; IM    
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MeSH Terms
Budd-Chiari Syndrome / etiology*,  pathology,  therapy
Hemoglobinuria, Paroxysmal / complications*
Liver / pathology

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

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