Camille Simard1, Marie-Lou Tardif2,3. 1. Department of Medicine, McGill University, Montreal, Canada. 2. Division of Obstetric Medicine, Department of Obstetrics and Gynecology, CHU Sainte-Justine, Montreal, Canada. 3. Department of Medicine, Université de Montréal, Montreal, Canada.
Abstract
Background: Hemophagocytic lymphohistiocytosis is a rare disorder, especially in pregnancy, characterized by excessive immune activation leading to hemophagocytic activity. Case Report: A 34-year-old woman presented at 31 weeks' gestation with fever, cytopenias, hyperferritinemia and fulminant hepatitis. Bone marrow biopsy was non-contributory, and no trigger was identified. Liver biopsy confirmed the diagnosis of hemophagocytic lymphohistiocytosis, and she received dexamethasone and etoposide, which was changed to anakinra to allow breastfeeding. Prompt diagnosis and treatment of hemophagocytic lymphohistiocytosis is crucial to the survival of pregnant women, but no consensus exists regarding the most appropriate therapy during pregnancy. Conclusion: Hemophagocytic lymphohistiocytosis is life-threatening and associated with significant morbidity and mortality. Important treatment considerations in pregnancy include maternal health, fetal prematurity and treatment toxicity. Anakinra has been successfully used in pregnancy and provides a promising alternative.
Background: Hemophagocytic lymphohistiocytosis is a rare disorder, especially in pregnancy, characterized by excessive immune activation leading to hemophagocytic activity. Case Report: A 34-year-old woman presented at 31 weeks' gestation with fever, cytopenias, hyperferritinemia and fulminant hepatitis. Bone marrow biopsy was non-contributory, and no trigger was identified. Liver biopsy confirmed the diagnosis of hemophagocytic lymphohistiocytosis, and she received dexamethasone and etoposide, which was changed to anakinra to allow breastfeeding. Prompt diagnosis and treatment of hemophagocytic lymphohistiocytosis is crucial to the survival of pregnant women, but no consensus exists regarding the most appropriate therapy during pregnancy. Conclusion: Hemophagocytic lymphohistiocytosis is life-threatening and associated with significant morbidity and mortality. Important treatment considerations in pregnancy include maternal health, fetal prematurity and treatment toxicity. Anakinra has been successfully used in pregnancy and provides a promising alternative.
Authors: Taryn Youngstein; Patrycja Hoffmann; Ahmet Gül; Thirusha Lane; Rene Williams; Dorota M Rowczenio; Huri Ozdogan; Serdal Ugurlu; John Ryan; Len Harty; Sean Riminton; Alex P Headley; Joachim Roesler; Norbert Blank; Jasmin B Kuemmerle-Deschner; Anna Simon; Adrian S Woolf; Philip N Hawkins; Helen J Lachmann Journal: Rheumatology (Oxford) Date: 2017-12-01 Impact factor: 7.580