Yasui M, Okamura T, Sakata N, Inoue M, Yagi K, Yoshimoto T, Mabuchi O, Sano M, Kawa K
Department of Pediatrics, Osaka Medical Center and Research Institute for Maternal and Child Health.
Rinsho Ketsueki. 2001 Nov;42(11):1111-6.
We report two boys with chronic active Epstein-Barr virus infection (CAEBV) refractory to conventional chemotherapy, who received HLA-mismatched allografts of CD34-positive progenitor cells from their fathers. One patient developed veno-occlusive disease (VOD) of the liver on day 18 after transplantation and died on day 26. The other patient received the allograft during partial remission. Although he suffered recurrent infections due to Streptococcus viridans, he is now doing well 23 months after transplantation. CAEBV refractory to chemotherapy is considered to be a fatal EBV-infected T/NK-cell lymphoproliferative disease, and our experiences suggest that CD34-positive progenitor cell transplantation for patients with CAEBV lacking HLA-matched donors may be a feasible and useful treatment. However, the timing of transplantation is considered to be critical, and should be performed when the patient is in good clinical condition.
我们报告了两名患有慢性活动性EB病毒感染（CAEBV）且对传统化疗无效的男孩，他们接受了来自父亲的HLA不匹配的CD34阳性祖细胞同种异体移植。一名患者在移植后第18天出现肝静脉闭塞性疾病（VOD），并于第26天死亡。另一名患者在部分缓解期接受了同种异体移植。尽管他因草绿色链球菌反复感染，但在移植后23个月时情况良好。对化疗难治的CAEBV被认为是一种致命的EB病毒感染的T/NK细胞淋巴增殖性疾病，我们的经验表明，对于缺乏HLA匹配供体的CAEBV患者，CD34阳性祖细胞移植可能是一种可行且有用的治疗方法。然而，移植时机被认为至关重要，应在患者临床状况良好时进行。
