Orphanet Journal of Rare Diseases (Jun 2021)

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

  • Lidong Liu,
  • Yutong Cui,
  • Qiongjie Zhou,
  • Huanqiang Zhao,
  • Xiaotian Li

DOI
https://doi.org/10.1186/s13023-021-01790-9
Journal volume & issue
Vol. 16, no. 1
pp. 1 – 10

Abstract

Read online

Abstract 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.

Keywords