Hepatic venoocclusive disease in blood and bone marrow transplantation in @entity1 and young adults: incidence, risk factors, and outcome in a cohort of 241 XXXX .

@entity1 @entity5 @entity648 @entity5885 @entity1576
PURPOSE: To describe recent characteristics of incidence, risk factors, treatment, and outcome of @entity5885 ( @entity5885 ) in @entity1 and young adults undergoing blood and bone marrow transplantation (BMT). METHODS: All @entity1 and young adults (n = 241) undergoing first myeloablative transplant at the UCSF Pediatric BMT unit between 1992 and 2000 were included. Retrospective chart review was done. Descriptive statistics and univariate and multivariate analyses of risk factors are presented. RESULTS: @entity5885 developed in 65 @entity1 (27%); it was severe in 13/65 @entity1 (20%). Matched unrelated donor transplantation, advanced-stage @entity5 , and transplantation in the recent period (1998-2000) were identified as significant risk factors for @entity5885 in univariate and multivariate analyses. Heparin prophylaxis did not decrease the incidence of @entity5885 . @entity5885 was diagnosed at a median day 8 after BMT. Five of 13 @entity1 with severe @entity5885 (38%) survived for more than 1 year after BMT, even after renal and @entity1576 and high total @entity648 levels up to 35 mg/dL. Nine of the 13 @entity1 received fibrinolytic treatment with tissue plasminogen activator, anti-thrombin 3, or defibrotide. The survival rate at day 100 after BMT for @entity1 with @entity5885 was 77%; it was 94% for those without @entity5885 . CONCLUSIONS: The persistently high incidence of @entity5885 , its significant impact on posttransplant survival, and the demonstration of recovery from even severe @entity5885 underscore the importance of early diagnosis and initiation of specific therapy. The use of Bearman's model of prediction of severity of @entity5885 and the application of fibrinolytic drugs when adequate are highly recommended.
