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2015 ; 2015
(ä): 876301
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Virus-associated hemophagocytic syndrome in renal transplant recipients: report
of 2 cases from a single center
#MMPMID25838952
Nanmoku K
; Yamamoto T
; Tsujita M
; Hiramitsu T
; Goto N
; Katayama A
; Narumi S
; Watarai Y
; Kobayashi T
; Uchida K
Case Rep Hematol
2015[]; 2015
(ä): 876301
PMID25838952
show ga
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.