Abstract
Virus-associated hemophagocytic syndrome (HPS) is a potentially fatal complication of immunosuppression for transplantation. However, it presents with heterogeneous clinical symptoms (fever, disturbed consciousness, and hepatosplenomegaly) and laboratory findings (pancytopenia, elevated hepatic enzyme levels, abnormal coagulation, and hyperferritinemia), impeding diagnosis. Case 1: A 39-year-old female developed fever 4 years after ABO-incompatible living-related renal transplantation. Laboratory findings revealed thrombocytopenia, elevated hepatic enzymes, Epstein-Barr virus (EBV) DNA seropositivity, and hyperferritinemia. EBV-associated HPS was confirmed by bone marrow aspiration. Steroid pulse therapy and etoposide were ineffective. Disseminated intravascular coagulation resulted in multiple organ failure, and the patient died 32 days after disease onset. Case 2: A 67-year-old male was admitted with rotavirus enteritis a month after living-unrelated renal transplantation. He developed sudden-onset high fever, disturbance of consciousness, and tachypnea 8 days after admission. Laboratory findings revealed elevated hepatic enzyme levels, hyperkalemia, and hyperferritinemia. Emergency continuous hemodiafiltration ameliorated the fever, and steroid pulse therapy improved abnormal laboratory values. Varicella-zoster virus meningitis was confirmed by spinal tap. Acyclovir improved consciousness, and he was discharged 87 days after admission. Fatal virus-associated HPS may develop in organ transplant patients receiving immunosuppressive therapy. Pathognomonic hyperferritinemia is useful for differential diagnosis.
Highlights
Hemophagocytic syndrome (HPS) is a life-threatening systemic inflammatory disease caused by infiltration of activated macrophages, which phagocytose blood cells in bone marrow, lymph nodes, and organs such as the liver and spleen [1]
We report 2 cases of virus-associated HPS in renal transplant recipients diagnosed as the result of hyperferritinemia
Virus-associated HPS was first described by Risdall et al in 1979 [2]
Summary
Hemophagocytic syndrome (HPS) is a life-threatening systemic inflammatory disease caused by infiltration of activated macrophages, which phagocytose blood cells in bone marrow, lymph nodes, and organs such as the liver and spleen [1]. The main triggers of reactive or secondary HPS are infectious diseases, viral infections [2]. Virus-associated HPS is a serious complication of immunocompromised conditions, such as drug-induced immunosuppression for organ transplantation [3]. The typical clinical findings of HPS are high fever, hepatosplenomegaly, and cytopenia. Characteristic symptoms may be absent in some patients, so the diagnosis is frequently missed [4]. We report 2 cases of virus-associated HPS in renal transplant recipients diagnosed as the result of hyperferritinemia
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