ABSTRACT
Hemophagocytic syndrome is a disorder of the mononuclear phagocytic system resulting in uncontrolled hemophagocytosis and cytokine overproduction. We report the first case of hemophagocytic syndrome, which occurred in a pregnantfemale patient 14 years after kidney transplant who displayed an atypical presentation and who had septic shock following cytomegalovirus infection. The patient, a-39-year-old woman at 27 weeks gestation with end-stage renal disease of unknown etiology, was admitted 14 years after living-donor kidney transplant (donor was her father) with high-grade fever, cough, and pancytopenia. Her immunosuppressant regimen included tacrolimus, azathioprine, and prednisone. Initially, she was hospitalized in the intensive care unit for septic shock without an identifiable focus of infection. She received intravenous broad-spectrum antibiotics before being transferred to our department following optimization of her hemodynamic status. Hemophagocytic syndrome was suspected, and bone marrow aspirate was performed, revealing macrophages with hemophagocytic activity. We confirmed the diagnosis of hemophagocytic syndrome given the presence of more than 5 criteria. We extensively investigated the underlying cause of hemophagocytic syndrome, and we diagnosed cytomegalovirus-induced hemophagocytic syndrome in a pregnant patient receiving immunosuppressive therapy after kidney transplantation. She was treated with corticosteroids and intravenous immunoglobulin. At 31 weeks gestation, she underwent a cesarean section; the baby developed newborn respiratory distress syndrome and died despite adequate resuscitation. We administered ganciclovir for 15 days following an increased cytomegalovirus viral load after delivery, leading to complete recovery.To date, optimal therapeutic and diagnostic guidelines for pregnancy-related hemophagocytic syndrome in female kidney transplant recipients are not well defined, and both patient and allograft survival rates remain low.
