Magro Cynthia M, Weinerman David J, Porcu Pierre Luigi, Morrison Carl D
Department of Pathology, Weill Medical College Cornell University, New York, NY 10021, USA.
J Cutan Pathol. 2007 Dec;34 Suppl 1:1-8. doi: 10.1111/j.1600-0560.2006.00712.x.
Post-transplant lymphoproliferative disease (PTLD) is a recognized complication of the immunosuppressive regimens associated with solid organ transplantation. The vast majority of these lesions represent monomorphic B-cell lymphoproliferative disease. Rarely, however, T-cell malignancies may emerge, the commonest being anaplastic large-cell lymphoma (ALCL).
We describe two patients who developed a post-transplant ALCL several years after transplantation. Comprehensive phenotypic and molecular studies were conducted. The technique of capillary gel electrophoresis was employed.
One patient died of unrelated causes, while the other patient did achieve clinical remission. The neoplastic cell populace was composed of CD4-positive cytotoxic T cells exhibiting CD30 positivity. There were very few B cells. Striking and prominent clonally restricted infiltrates were identified whereby there was both a heavy chain and T-cell beta gene rearrangement. There was no evidence of lytic Epstein-Barr virus (EBV) infection.
T-cell-associated PTLD does not appear to be directly attributable to EBV infection. Iatrogenic immune dysregulation may result in excessive T-cell proliferation to various antigenic stimuli, hence resembling other drug-associated cell lymphoproliferative conditions such as angioimmunoblastic lymphadenopathy. The dual rearrangement may have some implications regarding the cell of origin.
移植后淋巴细胞增殖性疾病（PTLD）是实体器官移植相关免疫抑制方案公认的并发症。这些病变绝大多数表现为单形性B细胞淋巴细胞增殖性疾病。然而，T细胞恶性肿瘤很少出现，最常见的是间变性大细胞淋巴瘤（ALCL）。
我们描述了两名患者，他们在移植后数年发生了移植后ALCL。进行了全面的表型和分子研究。采用了毛细管凝胶电泳技术。
一名患者死于无关原因，而另一名患者确实实现了临床缓解。肿瘤细胞群体由表现出CD30阳性的CD4阳性细胞毒性T细胞组成。B细胞很少。发现了显著且突出的克隆性受限浸润，存在重链和T细胞β基因重排。没有证据表明存在溶解性EB病毒（EBV）感染。
T细胞相关的PTLD似乎并非直接归因于EBV感染。医源性免疫失调可能导致T细胞对各种抗原刺激过度增殖，因此类似于其他药物相关的细胞淋巴细胞增殖性疾病，如血管免疫母细胞性淋巴结病。双重重排可能对起源细胞有一些影响。
