Kwong Y L, Lam C C, Chan T M
University Departments of Medicine and Pathology, Queen Mary Hospital, Hong Kong.
Br J Haematol. 2000 Jul;110(1):197-202. doi: 10.1046/j.1365-2141.2000.02138.x.
Post-transplantation lymphoproliferative disorders (PTLD) occur after solid organ and bone marrow transplantation. They are predominantly of B-cell and occasionally of T-cell lineage. We report a case of PTLD of natural killer (NK) cell lineage. A renal allograft recipient developed progressive pancytopenia 1 year after transplantation. Serial bone marrow biopsies showed an increasing infiltration by large granular lymphoid cells. A subsequent leukaemic phase also developed with systemic infiltration of other organs. Immunophenotyping showed that these cells were CD2+, CD3-, CD3epsilon+, CD56+, CD94+, CD158a- and CD158b-. In situ hybridization showed Epstein-Barr virus (EBV) infection of the neoplastic cells. Genotypical analysis showed the T-cell receptor gene in germline configuration and clonal EBV episomal integration. The overall features were consistent with NK cell lymphoma/leukaemia. The patient did not respond to cessation of immunosuppression or anti-EBV treatment. Combination chemotherapy was given, but the patient died ultimately of disseminated fungal infection. In conclusion, we have demonstrated that NK cell lymphoma is another rare type of PTLD that appears to be highly aggressive and therefore may require early chemotherapy to improve treatment outcome.
移植后淋巴细胞增生性疾病（PTLD）发生于实体器官和骨髓移植后。它们主要起源于B细胞，偶尔起源于T细胞谱系。我们报告一例自然杀伤（NK）细胞谱系的PTLD病例。一名肾移植受者在移植后1年出现进行性全血细胞减少。系列骨髓活检显示大颗粒淋巴细胞浸润增加。随后还发展为白血病期，其他器官出现全身浸润。免疫表型分析显示这些细胞CD2 +、CD3 -、CD3ε +、CD56 +、CD94 +、CD158a - 和CD158b -。原位杂交显示肿瘤细胞存在爱泼斯坦 - 巴尔病毒（EBV）感染。基因分型分析显示T细胞受体基因呈种系构型，EBV克隆性附加体整合。总体特征符合NK细胞淋巴瘤/白血病。患者对停用免疫抑制或抗EBV治疗无反应。给予联合化疗，但患者最终死于播散性真菌感染。总之，我们证明NK细胞淋巴瘤是另一种罕见的PTLD类型，似乎具有高度侵袭性，因此可能需要早期化疗以改善治疗效果。
