Post-transplant lymphoproliferative disorder (PTLD) is a rare, but severe Epstein-Barr virus (EPV)-driven disorder that manifest after hematopoietic stem cell transplantation (HSCT) or solid organ transplantation (SOT). This heterogenous disease may manifest as localized or disseminated, and clinical presentation may differ significantly. It may be difficult to early diagnose PTLD, as is may be misdiagnosed as infection or graft rejection. The majority of EBV-PTLD typically occurs within four months following HSCT, and almost all cases present within the first year. EBV-PTLD that manifests > 5 years is considered an exceedingly rare occurrence.

We describe a case of 66-year-old male, who was diagnosed with high-risk chronic lymphocytic leukemia (CLL). He underwent allogeneic HSCT from HLA-identical sister, and subsequently developed acute followed by chronic graft-versus-host disease, for which he was long-term treated with immunosuppressants. At 6 years following HSCT, the patient presented with life-threatening perforation of gut. Histological evaluation revealed diffuse large B cell lymphoma. Serum sample test showed positive EBV DNA and diagnosis of probable EBV-PTLD was done. After the treatment with rituximab, along with the reduction of immunosuppression, the patient achieved complete remission. Late onset EBV-PTLD after HSCT is extremely uncommon, and hardly described in literature.

中文翻译：

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异基因造血干细胞移植后患有慢性移植物抗宿主病的晚期爱泼斯坦-巴尔病毒相关的移植后淋巴细胞增生性疾病与肠道受累

移植后淋巴细胞增生性疾病（PTLD）是一种罕见但严重的由爱泼斯坦-巴尔病毒（EPV）驱动的疾病，在造血干细胞移植（HSCT）或实体器官移植（SOT）后出现。这种异质性疾病可能表现为局部或散播，临床表现可能有很大差异。早期诊断PTLD可能很困难，因为可能被误诊为感染或移植排斥。大多数EBV-PTLD通常发生在HSCT之后的四个月内，几乎所有病例都在第一年内出现。出现> 5年的EBV-PTLD被认为是极为罕见的。

我们描述了一个66岁的男性，被诊断出患有高风险的慢性淋巴细胞性白血病（CLL）的病例。他接受了与HLA相同的姐妹的同种异体HSCT，随后发展为急性，然后是慢性移植物抗宿主病，为此他长期接受了免疫抑制剂治疗。HSCT后6年，该患者出现了危及生命的肠穿孔。组织学评估显示弥漫性大B细胞淋巴瘤。血清样本测试显示EBV DNA阳性，并诊断出可能的EBV-PTLD。利妥昔单抗治疗后，随着免疫抑制的降低，患者完全缓解。HSCT后迟发性EBV-PTLD极为罕见，文献中几乎没有描述。