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- A Wahbi, J Graveleau, A Néel, M Joubert, A Masseau, G Magadur Joly, and M Hamidou.
- Service de médecine interne, CHU Hôtel-Dieu, 1, place Alexis-Ricordeau, 44093 Nantes cedex 1, France.
- Rev Med Interne. 2015 Aug 1;36(8):555-7.
IntroductionWe report a case of post-partum hemophagocytic lymphohistiocytosis with marked macrovesicular hepatic steatosis.Case ReportA 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.ConclusionSeveral 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.Copyright © 2014 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.
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