Background: Hemophagocytic syndrome is characterized by nonmalignant histiocytes that undergo uncontrolled phagocytosis of normal hematopoietic cells. Clinical severity ranges from complete recovery to rapid deterioration and death.
Case: Thrombocytopenia was discovered upon routine initial prenatal evaluation of a 24-year-old, gravida 2, para 1, at 29 weeks' gestation with a history of necrotizing lymphadenitis. Cytopenia and elevated transaminases developed, followed by hyperpyrexia. The patient delivered and her postpartum course was complicated by coagulopathy, multiorgan failure, and death. Bone marrow biopsy confirmed hemophagocytic syndrome.
Conclusion: Early diagnosis of hemophagocytic syndrome during pregnancy might be helped by recognizing symptoms and signs, including a history of necrotizing lymphadenitis, and obtaining a bone marrow biopsy.