title: [Macrovesicular hepatic steatosis revealing pregnancy hemophagocytic lymphohistiocytosis].
abstract: BACKGROUND
  We report a case of post-partum hemophagocytic lymphohistiocytosis with marked macrovesicular hepatic steatosis.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.affiliation: Service de médecine interne, CHU Hôtel-Dieu, 1, place Alexis-Ricordeau, 44093 Nantes cedex 1, France.
authors.forename: A
authors.initials: A
authors.lastname: Wahbi
authors.affiliation: Service de médecine interne, CHU Hôtel-Dieu, 1, place Alexis-Ricordeau, 44093 Nantes cedex 1, France.
authors.forename: J
authors.initials: J
authors.lastname: Graveleau
authors.affiliation: Service de médecine interne, CHU Hôtel-Dieu, 1, place Alexis-Ricordeau, 44093 Nantes cedex 1, France.
authors.forename: A
authors.initials: A
authors.lastname: Néel
authors.affiliation: Laboratoire d'anatomopathologie, CHU Hôtel-Dieu, 1, place Alexis-Ricordeau, 44093 Nantes cedex 1, France.
authors.forename: M
authors.initials: M
authors.lastname: Joubert
authors.affiliation: Service de médecine interne, CHU Hôtel-Dieu, 1, place Alexis-Ricordeau, 44093 Nantes cedex 1, France.
authors.forename: A
authors.initials: A
authors.lastname: Masseau
authors.affiliation: Service de médecine interne, centre hospitalier de Guingamp, 17, rue de l'Armor, BP 10548, 22205 Guingamp, France.
authors.forename: G Magadur
authors.initials: GM
authors.lastname: Joly
authors.affiliation: Service de médecine interne, CHU Hôtel-Dieu, 1, place Alexis-Ricordeau, 44093 Nantes cedex 1, France. Electronic address: mohamed.hamidou@chu-nantes.fr.
authors.forename: M
authors.initials: M
authors.lastname: Hamidou
mesh_terms: D000328:Adult; D005234:Fatty Liver / Q000209:etiology* / Q000473:pathology; D005260:Female; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000175:diagnosis; D011247:Pregnancy; D011644:Puerperal Disorders / Q000175:diagnosis
keywords: Fatty liver; Grossesse; Hemophagocytic lymphohistiocytis; Hemophagocytic syndrome; Postpartum; Pregnancy; Stéatose hépatique; Syndrome d’activation lymphohistiocytaire; Syndrome d’activation macrophagique