Please cite this article in press as: Yildiz H, et al. Hepatitis during pregnancy: A case of hemophagocytic lymphohistiocy- tosis. Clin Res Hepatol Gastroenterol (2017), https://doi.org/10.1016/j.clinre.2017.10.007 ARTICLE IN PRESS +Model CLINRE-1070; No. of Pages 7 Clinics and Research in Hepatology and Gastroenterology (2017) xxx, xxx.e1—xxx.e7 Available online at ScienceDirect www.sciencedirect.com CASE REPORT Hepatitis during pregnancy: A case of hemophagocytic lymphohistiocytosis Halil Yildiz a,* , Bernard Vandercam a , Xave Thissen a , Mina Komuta b , Nicolas Lanthier c , Frederic Debieve d , Geraldine Dahlqvist c a Department of internal medicine and infectiology, cliniques universitaires St Luc, 10, avenue hippocrate, 1200 Brussels, Belgium b Department of pathology, cliniques universitaires St Luc, 1200 Brussels, Belgium c Department of gastroenterology, cliniques universitaires St Luc, 1200 Brussels, Belgium d Department of gynecology and obstetric, cliniques universitaires St Luc, 1200 Brussels, Belgium KEYWORDS HIV; Pregnancy; Hepatitis; Hemophagocytic lymphohistiocytosis Summary Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe and potentially fatal syndrome that can occur during pregnancy. A 36 years-old woman, at 29 weeks of gestation, presented with itchiness and jaundice since a week. On clinical examination she was apyrexial and frankly icteric. Laboratory data showed evidence of acute hepatitis. A complete work-up was made excluding viral hepatitis (HAV, HEV, HBV, HCV, HHV6, CMV, EBV) and autoimmune liver disease. Liver diseases related to pregnancy were not completely excluded. A liver biopsy was performed and firstly interpreted as showing features of acute hepatitis. The clinical situation worsened, she developed fever with signs of fetal distress and emergent delivery was done. A second look at the liver biopsy showed features compatible with HLH, which was also confirmed on bone marrow biopsy. Extensive work-up with exclusion of infectious and malignant diseases, lead us to the diagnosis of HLH secondary to pregnancy and short term steroid therapy was started. She then completely recovered and didn’t present any relapse after 4 months of follow up. HLH during pregnancy is very rare and this is the first case of HLH presenting as acute hepatitis and diagnosed on a liver biopsy. © 2017 Elsevier Masson SAS. All rights reserved. ∗ Corresponding author. E-mail address: halil.yildiz@uclouvain.be (H. Yildiz). https://doi.org/10.1016/j.clinre.2017.10.007 2210-7401/© 2017 Elsevier Masson SAS. All rights reserved.