title: Virus-associated hemophagocytic syndrome in renal transplant recipients: report of 2 cases from a single center.
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.
authors.affiliation: Surgical Branch, Institute of Kidney Diseases, Jichi Medical University Hospital, 3311-1 Yakushiji, Shimotsuke City, Tochigi 329-0498, Japan.
authors.forename: Koji
authors.identifier: 0000-0003-3987-4832
authors.initials: K
authors.lastname: Nanmoku
authors.affiliation: Department of Transplant Surgery, Nagoya Daini Red Cross Hospital, Nagoya, Japan.
authors.forename: Takayuki
authors.initials: T
authors.lastname: Yamamoto
authors.affiliation: Department of Transplant Surgery, Nagoya Daini Red Cross Hospital, Nagoya, Japan.
authors.forename: Makoto
authors.initials: M
authors.lastname: Tsujita
authors.affiliation: Department of Transplant Surgery, Nagoya Daini Red Cross Hospital, Nagoya, Japan.
authors.forename: Takahisa
authors.initials: T
authors.lastname: Hiramitsu
authors.affiliation: Department of Transplant Surgery, Nagoya Daini Red Cross Hospital, Nagoya, Japan.
authors.forename: Norihiko
authors.initials: N
authors.lastname: Goto
authors.affiliation: Department of Transplant Surgery, Masuko Memorial Hospital, Nagoya, Japan.
authors.forename: Akio
authors.initials: A
authors.lastname: Katayama
authors.affiliation: Department of Transplant Surgery, Nagoya Daini Red Cross Hospital, Nagoya, Japan.
authors.forename: Shunji
authors.initials: S
authors.lastname: Narumi
authors.affiliation: Department of Transplant Surgery, Nagoya Daini Red Cross Hospital, Nagoya, Japan.
authors.forename: Yoshihiko
authors.identifier: 0000-0002-7104-3118
authors.initials: Y
authors.lastname: Watarai
authors.affiliation: Department of Transplant Immunology, Nagoya University School of Medicine, Nagoya, Japan.
authors.forename: Takaaki
authors.initials: T
authors.lastname: Kobayashi
authors.affiliation: Department of Organ Transplant Surgery, Aichi Medical University Hospital, Nagakute, Japan.
authors.forename: Kazuharu
authors.initials: K
authors.lastname: Uchida
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