Perioperative Management of Recurrent Hemophagocytic Syndrome in a Pregnant Woman: A Case Report

Abstract

BACKGROUND Hemophagocytic syndrome (HPS) is a rare syndrome characterized by abnormal activation of histiocytes and hemophagocytosis. We report the clinical management of recurrent HPS following 2 cesarean sections in the same patient. CASE REPORT A 33-year-old primiparous mother presented during her second trimester of pregnancy, and HPS was diagnosed based on pancytopenia, hyperferritinemia (13 170 ng/ml), and hemophagocytosis in bone marrow examination. Despite steroid therapy, her HPS did not improve. Following the delivery of a healthy premature infant, there was no improvement in HPS, and immunochemotherapy was started 4 days postoperatively. Thrombocytopenia and hyperferritinemia persisted but normalized over the next 2 months, and immunochemotherapy was discontinued after 6 months. About 1 year after chemotherapy, the patient became pregnant with her second child. At 35 weeks of gestation, recurrence of HPS was suspected, and a C-section was performed at 36 weeks of gestation. The surgery was complicated by placenta previa, and general anesthesia was initiated after successful delivery of the infant. Epidural anesthesia was not performed due to concerns for postoperative thrombocytopenia. CONCLUSIONS Interestingly, HPS was likely triggered twice by pregnancy in this patient. Although reports of HPS during pregnancy are rare, there have been reports of rapid deterioration and death. Early diagnosis and therapeutic intervention are essential.

Figure 1.

Patient presentation and treatment course during the first pregnancy. At 28 weeks of gestation, HPS was diagnosed, and steroid therapy was initiated. Hepatic dysfunction improved, but serum ferritin did not decrease, platelet count dropped, and fever persisted. To prevent maternal complications, premature delivery of the fetus and a cesarean section were performed at 29 weeks. However, after delivery, HPS did not improve, and immunochemotherapy with dexamethasone, etoposide, and cyclosporine was started on the fourth postoperative day. After the start of chemotherapy, the patient’s fever gradually resolved, and her platelet count increased. The patient was discharged from the hospital after her platelet count normalized 1 month following delivery. Ferritin was normalized 2 months after surgery, and chemotherapy was completed 6 months after delivery.

Figure 2.

Patient presentation and treatment course during the second pregnancy. The patient presented with fever and elevated ferritin (3483.2 ng/ml) at 35 weeks of gestation and was diagnosed with recurrent HPS and scheduled for cesarean section at 36 weeks of gestation. Postoperatively, anemia with Hb 7.3 g/dl and decreased platelets (126 000/µl) were observed, but with follow-up, they improved on the 5^th postoperative day, and no coagulopathy or liver dysfunction was observed. Ferritin showed a decreasing trend after delivery, and at 2 months postoperatively it was elevated but stable at 508 ng/ml.