A Rare Case of Hemophagocytic Lymphohistiocytosis in Pregnancy With Clinical Features Resembling Acute Fatty Liver of Pregnancy

Hemophagocytic lymphohistiocytosis (HLH) during pregnancy is a rare but critical condition that is difficult to diagnose due to its complex and nonspecific presentation. We report a case of HLH in a 27-year-old woman at 32 weeks of gestation. She presented with persistent fever and liver dysfunction, leading to a suspected diagnosis of HELLP syndrome and an emergency cesarean section. However, her condition deteriorated after delivery, with the development of pancytopenia, coagulopathy, hemophagocytosis, and fatty liver. These findings raised suspicion of HLH or acute fatty liver of pregnancy (AFLP). A comprehensive diagnostic workup, including liver biopsy and genetic testing, confirmed the diagnosis of HLH. HLH during pregnancy often mimics obstetric complications such as AFLP, underscoring the importance of careful differential diagnosis.