Postpartum hemophagocytic lymphohistiocytosis: A case report

BACKGROUND: Postpartum hemophagocytic lymphohistiocytosis (HLH) is a rare disease with unclear pathophysiology. It is a secondary HLH diagnosed using the pediatric diagnostic criteria; however, the clinical diagnosis of postpartum HLH remains challenging. Hence, HLH may remain undiagnosed, leading to poor patient prognosis. Therefore, improvements in the accuracy of postpartum HLH diagnoses and treatments are necessary. CASE SUMMARY: We report the case of a 40-year-old female with postpartum HLH. The patient attended the postpartum care center for 3 wk after giving birth and underwent needle aspiration due to thyroid gland enlargement 11 d before an emergency department visit precipitated by fever and abdominal pain. Since no abnormal emergency room findings were noted, the patient was discharged with a prescription for broad-spectrum antibiotics. Three days later, she returned to the emergency room in a hemodynamically unstable state and was admitted to the intensive care unit with suspected sepsis or hematologic disease. The patient was treated, without effect, for sepsis using broad-spectrum antibiotics, and for suspected hematologic disease with steroid therapy. However, she died due to rapidly worsening symptoms. CONCLUSION: Rapid recognition and appropriate treatment of postpartum HLH are needed to improve the prognosis.