Document Detail


Hepatic manifestations of hemophagocytic syndrome: a study of 30 cases.
MedLine Citation:
PMID:  11280564     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: Hemophagocytic syndrome has been defined as the combination of a proliferation of cytologically benign, actively phagocytic macrophages in bone marrow, spleen, or lymph nodes in association with fever, cytopenia, splenomegaly, and hypertriglyceridemia. Hepatic dysfunction is often present but the nature of the hepatic lesions and related manifestations have not been fully characterized. The aim of this study was to ascertain the features of hepatic involvement in hemophagocytic syndrome. METHODS: Thirty patients with hemophagocytic syndrome were retrospectively studied. Inclusion criteria included: 1) bone marrow with hemophagocytic histiocytosis, 2) clinical or biological signs of hepatic involvement, and 3) available liver specimen. RESULTS: The association of fever, jaundice, and hepatomegaly or splenomegaly was present in 50% of the patients. Median value for serum alanine transaminase activity was five times the upper limit of normal values (range, 0.3-125), for serum alkaline phosphatase activity 2.7 upper limit of normal values (range, 0.2-47.7), for total bilirubin 136 micromol/L (range, 4-681 micromol/L), and for factor V 70% (range, 19-145%). Sinusoidal dilatation with hemophagocytic histiocytosis were found in the biopsy specimen in all patients. An underlying condition potentially responsible for altered immune function (lymphoma, leukemia, liver transplantation) was identified in 29 patients. Liver biopsy was diagnostic for the underlying condition in 15 patients (including eight cases with nonspecific bone marrow biopsy findings). High serum bilirubin, elevated serum alkaline phosphatase activity, low factor V level, and lack of treatment for the underlying disease were associated with a poor prognosis. CONCLUSIONS: Hemophagocytic syndrome should be suspected in immunodeficient patients with fever, jaundice, and hepatosplenomegaly. Hepatic lesions are characterized by nonspecific sinusoidal dilatation with hemophagocytic histiocytosis and in 50% of the patients by alterations specific to the underlying condition. Liver biopsy is a useful diagnostic procedure in patients with this clinical presentation.
Authors:
C de Kerguenec; S Hillaire; V Molinié; C Gardin; C Degott; S Erlinger; D Valla
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The American journal of gastroenterology     Volume:  96     ISSN:  0002-9270     ISO Abbreviation:  Am. J. Gastroenterol.     Publication Date:  2001 Mar 
Date Detail:
Created Date:  2001-03-30     Completed Date:  2001-04-26     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0421030     Medline TA:  Am J Gastroenterol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  852-7     Citation Subset:  IM    
Affiliation:
Service d'Hépatologie, H pital Beaujon, Clichy, France.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Aged
Aged, 80 and over
Female
Histiocytosis, Non-Langerhans-Cell / complications*
Humans
Liver / pathology,  physiopathology
Liver Diseases / etiology*,  pathology,  physiopathology
Male
Middle Aged
Retrospective Studies
Survival Analysis

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


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