McCarthy M, Ramage J, McNair A, Gane E, Portmann B, Pagliuca A, Rela M, Heaton N, Mufti G J, Williams R
Institute of Liver Studies, King's College Hospital and King's College School of Medicine and Dentistry, London, UK.
J Hepatol. 1997 Dec;27(6):1015-21. doi: 10.1016/s0168-8278(97)80145-6.
BACKGROUND/AIMS: Post-transplant lymphoproliferative disorder is a well-documented complication with an incidence ranging from 2 to 10%, depending on the organ transplanted. Yet despite our increased understanding of the pathophysiology of this disease and the various treatments available, the mortality remains high at 60-80%. We present the clinical and histological features of ten adult liver transplant recipients with post-transplant lymphoproliferative disorder presenting over a 15-year period and review the therapeutic options.
CD20/CD45RO immunostaining was used for T/B-cell markers; polymerase chain reaction and in-situ hybridisation for Epstein-Barr virus genome detection; kappa/lambda immunostaining and gene rearrangement analysis for clonality.
There were six females and four males (age range 24-56) with onset of post-transplant lymphoproliferative disorder-symptoms ranging from 3 to 72 months post transplant. Sites of post-transplant lymphoproliferative disorder included liver (n=4), lymph nodes (n=5), bone marrow (n=2), lungs (n=2), kidneys (n=2), brain, ovaries,: and pancreas (n=1). All lesions were classified as high-grade lymphoma, of B-cell lineage (9 tested); Epstein-Barr virus genome was detected in 7/10 cases. Three tumours were monoclonal; four were polyclonal and three undetermined. Treatment included immunosuppression reduction, antiviral therapy with acyclovir and/or chemotherapy (CHOP or VAPEC-B). Survival times for those patients not treated with chemotherapy were from 9 days to 30 months, whereas those receiving chemotherapy had remission times of 4 to 48 months.
Longer-term remissions can be achieved in patients treated with systemic chemotherapy, although not without morbidity. Clonality assessment is important but treatment decisions should be based primarily on clinical features of progression, as polyclonal tumours can behave as aggressively as monoclonal tumours.
背景/目的：移植后淋巴细胞增生性疾病是一种有充分文献记载的并发症，其发病率在2%至10%之间，具体取决于所移植的器官。然而，尽管我们对该疾病的病理生理学以及现有的各种治疗方法有了更多了解，但其死亡率仍高达60%至80%。我们呈现了15年间10例成年肝移植受者发生移植后淋巴细胞增生性疾病的临床和组织学特征，并回顾了治疗选择。
采用CD20/CD45RO免疫染色检测T/B细胞标志物；采用聚合酶链反应和原位杂交检测爱泼斯坦-巴尔病毒基因组；采用kappa/lambda免疫染色和基因重排分析检测克隆性。
6例女性和4例男性（年龄范围24至56岁）发生移植后淋巴细胞增生性疾病，症状出现在移植后3至72个月。移植后淋巴细胞增生性疾病的发病部位包括肝脏（4例）、淋巴结（5例）、骨髓（2例）、肺（2例）、肾（2例）、脑、卵巢和胰腺（1例）。所有病变均被分类为B细胞系高级别淋巴瘤（9例检测）；10例中有7例检测到爱泼斯坦-巴尔病毒基因组。3个肿瘤为单克隆性；4个为多克隆性，3个未确定。治疗包括减少免疫抑制、用阿昔洛韦进行抗病毒治疗和/或化疗（CHOP或VAPEC - B）。未接受化疗的患者生存时间为9天至30个月，而接受化疗的患者缓解时间为4至48个月。
接受全身化疗的患者可实现较长时间的缓解，尽管并非没有并发症。克隆性评估很重要，但治疗决策应主要基于病情进展的临床特征，因为多克隆肿瘤的行为可能与单克隆肿瘤一样具有侵袭性。
