Document Detail

Nodular regenerative hyperplasia: the main liver disease in patients with primary hypogammaglobulinemia and hepatic abnormalities.
MedLine Citation:
PMID:  17998147     Owner:  NLM     Status:  MEDLINE    
BACKGROUND/AIMS: Liver lesions associated with primary hypogammaglobulinemia have been poorly described. We aimed to assess the clinical, histological and immune features and outcome of hepatic injury in patients with primary hypogammaglobulinemia. METHODS: The medical records of 51 patients (23 patients with liver biopsy) with primary hypogammaglobulinemia and liver abnormalities were retrospectively reviewed. Forty-three controls with primary hypogammaglobulinemia but with no hepatic manifestations were analyzed in parallel. RESULTS: Cholestasis (65%), mainly anicteric, and portal hypertension (50%) were the main hepatic manifestations. Histological analysis revealed non-fibrosing architectural abnormalities consistent with nodular regenerative hyperplasia (NRH) in 84% of CVID patients and in all HIGM and XLA patients. Intrasinusoidal lymphocytic infiltration, abnormalities of portal vessels and epithelioid granulomas were observed in 90%, 43% and 44% of patients, respectively. NRH was associated with portal hypertension in 75% of the cases. These patients more often presented with autoimmune diseases and peripheral lymphocytic abnormalities than control patients (p < 0.05). CONCLUSIONS: Liver involvement in primary hypogammaglobulinemia mainly consists of NRH leading to chronic cholestasis and portal hypertension. Association with intrasinusoidal T cell infiltration, portal vein endotheliitis, autoimmune diseases and peripheral lymphocytic abnormalities suggests an autoimmune mechanism.
Georgia Malamut; Marianne Ziol; Felipe Suarez; Michel Beaugrand; Jean François Viallard; Anne Sophie Lascaux; Virginie Verkarre; Dominique Bechade; Thierry Poynard; Olivier Hermine; Christophe Cellier
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Publication Detail:
Type:  Journal Article     Date:  2007-10-17
Journal Detail:
Title:  Journal of hepatology     Volume:  48     ISSN:  0168-8278     ISO Abbreviation:  J. Hepatol.     Publication Date:  2008 Jan 
Date Detail:
Created Date:  2007-12-10     Completed Date:  2008-03-11     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8503886     Medline TA:  J Hepatol     Country:  England    
Other Details:
Languages:  eng     Pagination:  74-82     Citation Subset:  IM    
Hepatology and Gastroenterology Department, Hôpital Européen Georges Pompidou, Paris, France.
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MeSH Terms
Agammaglobulinemia / complications*,  pathology
Age of Onset
Autoantibodies / analysis
Common Variable Immunodeficiency / complications
Enzymes / blood
Hyper-IgM Immunodeficiency Syndrome / complications
Liver / abnormalities*,  pathology*
Liver Circulation
Liver Diseases / epidemiology,  etiology*,  pathology
Liver Function Tests
Lymphocytes / immunology
Middle Aged
Portal Vein / abnormalities,  pathology
Sex Ratio
Treatment Outcome
Reg. No./Substance:
0/Autoantibodies; 0/Enzymes

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