[Macrovesicular hepatic steatosis revealing pregnancy hemophagocytic lymphohistiocytosis].

Abstract

INTRODUCTION:

We report a case of post-partum hemophagocytic lymphohistiocytosis with marked macrovesicular hepatic steatosis.

CASE REPORT:

A 39-year-old woman was admitted for hemophagocytic lymphohistiocytosis with a serum ferritin level of 103,380 μg/L. Thoracic abdominal and pelvic CT-scan showed hepatomegaly with marked steatosis. Liver biopsy confirmed macrovesicular steatosis. The diagnosis was a primary hemophagocytic lymphohistiocytosis. After treatment failure including corticosteroids, intravenous immunoglobulin, tetracycline, acyclovir, antituberculosis drugs, and anti-IL1R therapy, clinical improvement was obtained with intravenous cyclosporine. At 4-year follow-up, the patient remained asymptomatic.

CONCLUSION:

Several aspects of this report of primary hemophagocytic lymphohistiocytosis are remarkable and include the association with post-partum, the severe radiologic and histologic macrovesicular steatosis, and the dramatic efficacy of cyclosporine.

• Authors: A Wahbi, J Graveleau, A Néel, M Joubert, A Masseau, G Magadur Joly, M Hamidou
• Journal: La Revue de medecine interne (Rev Med Interne) Vol. 36 Issue 8 Pg. 555-7 (Aug 2015) ISSN: 1768-3122 [Electronic] France
• Vernacular Title: Stéatose hépatique macrovésiculaire majeure révélant un syndrome d'activation lymphohistiocytaire postgravidique.
• PMID: 25304169 (Publication Type: Case Reports, English Abstract, Journal Article)
• Copyright: Copyright © 2014 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

Topics

• Adult
• Fatty Liver (etiology, pathology)
• Female
• Humans
• Lymphohistiocytosis, Hemophagocytic (complications, diagnosis)
• Pregnancy
• Puerperal Disorders (diagnosis)