Kunitomi Akane, Kimura Hiroshi, Ito Yoshinori, Naitoh Kazuyuki, Noda Nobuhiko, Iida Hiroatsu, Sao Hiroshi
Department of Hematology, Meitetsu Hospital, Nagoya, Japan.
J Clin Exp Hematop. 2011;51(1):57-61. doi: 10.3960/jslrt.51.57.
We present a case of life-threatening Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (HLH) with severe hepatitis that was successfully treated by allogeneic stem cell transplantation from an unrelated donor. A 26-year-old woman was admitted to hospital with a high fever and liver dysfunction. Laboratory tests, including bone marrow aspiration, revealed severe HLH that occurred after EBV infection. High-dose methylprednisolone and etoposide therapy did not control the disease. We could control the HLH, but the EBV viremia continued following the CHOPE (cyclophosphamide, doxorubicin, vincristine, prednisone, and etoposide) chemotherapy regimen. Therefore, the patient underwent allogeneic bone marrow transplantation from an HLA-matched, unrelated donor. The patient has remained in good condition without disease recurrence for 2 years after bone marrow transplantation. Although there is no consensus regarding allogeneic stem cell transplantation for EBV-HLH, it is the treatment of choice for aggressive EBV-HLH when the patient is refractory to intensive chemotherapy.
我们报告一例危及生命的与 Epstein-Barr 病毒（EBV）相关的噬血细胞性淋巴组织细胞增生症（HLH）合并严重肝炎的病例，该患者通过接受来自无关供体的异基因干细胞移植成功治愈。一名 26 岁女性因高热和肝功能不全入院。包括骨髓穿刺在内的实验室检查显示，该患者在 EBV 感染后发生了严重的 HLH。大剂量甲泼尼龙和依托泊苷治疗未能控制病情。我们虽能控制 HLH，但在采用 CHOPE（环磷酰胺、阿霉素、长春新碱、泼尼松和依托泊苷）化疗方案后，EBV 病毒血症仍持续存在。因此，该患者接受了来自 HLA 匹配的无关供体的异基因骨髓移植。骨髓移植后 2 年，患者一直状况良好，无疾病复发。尽管对于 EBV-HLH 的异基因干细胞移植尚无共识，但当患者对强化化疗耐药时，它是侵袭性 EBV-HLH 的首选治疗方法。
