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Hemophagocytic lymphohistiocytosis during pregnancy is rare; it is often misdiagnosed, resulting in a high maternal and foetal mortality rate. Herein, based on limited case reports including antepartum and postpartum cases, we reviewed the current studies of pregnancy-related hemophagocytic lymphohistiocytosis, and compared the epidemiology, aetiology, diagnosis and treatment of pregnancy-related hemophagocytic lymphohistiocytosis with non-pregnancy, enriching the understanding of hemophagocytic lymphohistiocytosis and its treatment in obstetrics.   Supplementary Information  The online version contains supplementary material available at 10.1186/s13023-021-01790-9.

Hemophagocytic lymphohistiocytosis during pregnancy: a review of the literature in epidemiology, pathogenesis, diagnosis and treatment