Document Detail


Kasabach-Merrit syndrome and adult hepatic epithelioid hemangioendothelioma an unusual association.
MedLine Citation:
PMID:  15664259     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Kasabach-Merrit syndrome (KMS) is very rarely observed in adults associated with visceral hemangiomas. Hepatic epithelioid hemangioendothelioma (HEHE) is a very rare clinical entity with an intermediate malignant potential and a mortality rate of 20-30%. We described a case of KMS associated with HEHE in a 70 year old male patient who presented coagulation abnormalities, low platelet count and high fibrin degradation products that were typical features of KMS. Histopathology of the hepatic tissue and a positive immunostaining for endothelial markers confirmed the diagnosis of an HEHE. The first description of KMS was made in children in 1940. It was described as a "consumptive coagulopathy with capillary hemangiomas". However, recent reports have shown that this condition could be associated with kaposiform hemangioendotheliomas, an aggressive form of giant hemangioma. A giant hemangioma in adults as well as an hemangioendothelioma in children could be associated KMS, but an HEHE has been rarely reported in association with this syndrome in adult patients.
Authors:
Bernardo Frider; Andrés Bruno; Julio Selser; Rodríguez Vanesa; Pablo Pascual; Raquel Bistoletti
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of hepatology     Volume:  42     ISSN:  0168-8278     ISO Abbreviation:  J. Hepatol.     Publication Date:  2005 Feb 
Date Detail:
Created Date:  2005-01-24     Completed Date:  2005-08-02     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8503886     Medline TA:  J Hepatol     Country:  England    
Other Details:
Languages:  eng     Pagination:  282-3     Citation Subset:  IM    
Affiliation:
Division of Internal Medicine-Hepatology, Hospital General de Agudos Dr Cosme Argerich, Ciudad Autónoma de Buenos Aires, Alte Brown 240, (1155), Buenos Aires, Argentina. bernardo@frider.com.ar
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MeSH Terms
Descriptor/Qualifier:
Aged
Biopsy
Hemangioendothelioma / complications,  pathology*
Humans
Liver / pathology*
Liver Diseases / complications,  pathology*
Male
Platelet Count
Syndrome

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


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