Post‐transplantation lymphoproliferative disease of natural killer cell lineage: a clinicopathological and molecular analysis
Post‐transplantation lymphoproliferative disease of natural killer cell lineage: a clinicopathological and molecular analysis
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DOI：
10.1046/j.1365-2141.2000.02138.x
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发表时间：
2000-07
影响因子：
6.5
通讯作者：
Y. Kwong;CK Lam;T. M. Chan
中科院分区：
文献类型：
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作者：
Y. Kwong;CK Lam;T. M. Chan
移植后淋巴组织增生性疾病（PTLD）发生在实体器官和骨髓移植后。它们主要是B细胞，偶尔是T细胞谱系。我们报告一例自然杀伤细胞系的PTLD。一例肾移植受者在移植后1年发生进行性全血细胞减少症。连续骨髓活检显示大颗粒淋巴样细胞浸润增加。随后的白血病阶段也发展为其他器官的全身性浸润。免疫表型分析显示这些细胞为CD 2+、CD 3 −、CD 3 ε+、CD 56+、CD 94+、CD 158 a −和CD 158 b −。原位杂交显示肿瘤细胞的Epstein巴尔病毒（EBV）感染。基因型分析显示T细胞受体基因在生殖系构型和克隆EBV附加体整合中。总体特征与NK细胞淋巴瘤/白血病一致。患者对停止免疫抑制或抗EBV治疗无应答。给予联合化疗，但患者最终死于播散性真菌感染。总之，我们已经证明，NK细胞淋巴瘤是另一种罕见的PTLD类型，似乎是高度侵袭性的，因此可能需要早期化疗，以改善治疗结果。
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−, CD3ε+, 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.