Yui Shunsuke, Yamaguchi Hiroki, Imadome Ken-ichi, Arai Ayako, Takahashi Mikiko, Ohashi Ryuji, Tamai Hayato, Moriya Keiichi, Nakayama Kazutaka, Shimizu Akira, Inokuchi Koiti
Department of Hematology, Nippon Medical School.
J Nippon Med Sch. 2016;83(1):35-42. doi: 10.1272/jnms.83.35.
We report a case of the extremely rare condition Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disease (LPD) which occurred after umbilical cord blood transplantation. A 25-year-old Japanese man underwent cord blood transplantation from a male human leukocyte antigen 4/6-matched donor due to acute myeloid leukemia with trisomy 8. Bone marrow examination on day 30 showed chimerism with at least 90% donor cells and complete hematological response. Chronic symptoms of graft-versus-host disease appeared only on the skin and were successfully treated with cyclosporine alone. Three years later, however, the patient experienced repeated cold-like symptoms and was hospitalized with liver dysfunction. A high fever developed and was followed by significant edema of the right side of the face. The EBV DNA copy number in whole peripheral blood was 2×10(4)/mL. Liver biopsy showed invasion of EBV-infected CD8-positive T cells. Southern blotting analysis of the whole peripheral blood showed that the T-cell receptor Cβ1 rearrangement was positive. On the basis of these results, EBV-positive T-cell LPD was diagnosed and treated with prednisolone, cyclosporine, and etoposide, followed by cyclophosphamide, doxorubicin, vincristine, and prednisone. However, the patient died of cardiac function failure, pneumonia, and pulmonary hemorrhage, all of unidentified cause. Most cases of EBV-related LPD after hematopoietic stem cell transplantation consist of EBV-positive B-cell LPD, and, to our knowledge, de novo EBV-positive T-cell LPD subsequent to transplantation has not been previously reported.
我们报告一例极为罕见的脐带血移植后发生的爱泼斯坦-巴尔病毒（EBV）阳性T细胞淋巴增殖性疾病（LPD）病例。一名25岁的日本男性因急性髓系白血病伴8号染色体三体，接受了来自男性人类白细胞抗原4/6配型相合供者的脐带血移植。移植后第30天的骨髓检查显示嵌合体中供者细胞至少占90%，且血液学完全缓解。移植物抗宿主病的慢性慢性症状仅出现在皮肤上，单独使用环孢素治疗成功。然而，三年后，患者反复出现类似感冒的症状，并因肝功能障碍住院。随后出现高热，继而右侧面部明显水肿。全外周血中EBV DNA拷贝数为2×10⁴/mL。肝脏活检显示EBV感染的CD8阳性T细胞浸润。全外周血的Southern印迹分析显示T细胞受体Cβ1重排呈阳性。基于这些结果，诊断为EBV阳性T细胞LPD，并给予泼尼松龙、环孢素和依托泊苷治疗，随后给予环磷酰胺、阿霉素、长春新碱和泼尼松治疗。然而，患者死于心功能衰竭、肺炎和肺出血，病因均不明。造血干细胞移植后大多数EBV相关LPD病例为EBV阳性B细胞LPD，据我们所知，移植后新发的EBV阳性T细胞LPD此前尚未见报道。
