La Revue de medecine interne · 2014
[Macrovesicular hepatic steatosis revealing pregnancy hemophagocytic lymphohistiocytosis].
Клинический случайАннотация на английскомСтатьяНазвание и аннотация: Перевод в очереди
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Wahbi A, Graveleau J, Néel A, Joubert M, Masseau A, Joly G Magadur, Hamidou M
Аннотация
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.
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.