Hemophagocytic lymphohistiocytosis presenting with nonimmune hydrops fetalis.

Hydrops fetalis is a condition of diverse etiologies. An association between hemophagocytic lymphohistiocytosis (HLH) and hydrops fetalis has not been reported in the English literature. We describe an intrauterine case of HLH in an infant who presented with hydrops fetalis at 32 weeks gestation. We suggest that HLH should be considered in the differential diagnosis of nonimmune hydrops fetalis, especially in the presence of cytopenias.