Nanmoku Koji, Yamamoto Takayuki, Tsujita Makoto, Hiramitsu Takahisa, Goto Norihiko, Katayama Akio, Narumi Shunji, Watarai Yoshihiko, Kobayashi Takaaki, Uchida Kazuharu
Surgical Branch, Institute of Kidney Diseases, Jichi Medical University Hospital, 3311-1 Yakushiji, Shimotsuke City, Tochigi 329-0498, Japan.
Department of Transplant Surgery, Nagoya Daini Red Cross Hospital, Nagoya, Japan.
Case Rep Hematol. 2015;2015:876301. doi: 10.1155/2015/876301. Epub 2015 Mar 8.
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.
病毒相关性噬血细胞综合征（HPS）是移植免疫抑制的一种潜在致命并发症。然而，其临床症状（发热、意识障碍和肝脾肿大）及实验室检查结果（全血细胞减少、肝酶水平升高、凝血异常和高铁蛋白血症）具有异质性，这给诊断带来了困难。病例1：一名39岁女性在ABO血型不相容的活体亲属肾移植4年后出现发热。实验室检查发现血小板减少、肝酶升高、EB病毒（EBV）DNA血清学阳性及高铁蛋白血症。骨髓穿刺确诊为EBV相关性HPS。类固醇冲击治疗和依托泊苷治疗无效。弥散性血管内凝血导致多器官功能衰竭，患者在发病32天后死亡。病例2：一名67岁男性在非亲属活体肾移植1个月后因轮状病毒肠炎入院。入院8天后突然出现高热、意识障碍和呼吸急促。实验室检查发现肝酶水平升高、高钾血症和高铁蛋白血症。紧急连续性血液透析滤过改善了发热症状，类固醇冲击治疗使异常实验室检查值恢复正常。腰椎穿刺确诊为水痘-带状疱疹病毒脑膜炎。阿昔洛韦改善了意识，患者在入院87天后出院。接受免疫抑制治疗的器官移植患者可能会发生致命的病毒相关性HPS。具有诊断意义的高铁蛋白血症有助于鉴别诊断。
