肾移植后晚发型淋巴组织增生性疾病的临床病理特点
发布时间：2018-11-21 09:00
【摘要】：目的移植后淋巴组织增生性疾病(post-transplant lymphoproliferative disorders,PTLD)是实体器官移植或造血干细胞移植后的一种严重并发症,主要与医源性免疫抑制和Epstein-Barr病毒(EBV)感染有关。由于发生率低,临床表现和病理形态多样,前瞻性临床研究少,目前对该病的认识还很不充分。本研究旨在探讨肾移植后晚发型PTLD的临床病理特点及治疗和预后。方法 2001-01-30-2013-12-30在南京军区南京总医院根据WHO造血与淋巴组织肿瘤分类标准共确诊4例肾移植后PTLD,回顾性分析该组患者的临床病理资料、EBV相关检查结果及治疗和预后,并对相关文献进行复习。结果 4例患者肾移植后均采用三联或四联免疫抑制治疗,肾移植至PTLD诊断时间为2.5~18年(中位9.5年)。4例的病理类型均为单形型,其中1例非特指型外周T细胞淋巴瘤(PTCL-NOS)和3例非生发中心型弥漫大B细胞淋巴瘤(DLBCL)。外周血EBV-DNA和病理组织原位杂交检测EBER均阴性。1例PTCL-NOS以皮肤病变起病,合并噬血细胞淋巴组织细胞增生症,3例DLBCL分别以颌下肿块、腰背痛和下肢无力起病。确诊后均予免疫抑制剂减量,同时行联合化疗或利妥昔单抗联合化疗。4例患者中,2例早期死亡,分别为疾病进展和化疗后并发感染性休克。另2例分别在完全缓解4个月和10个月后疾病复发,再次治疗未缓解。结论肾移植后晚发型PTLD以单形型为主,DLBCL最常见,结外侵犯多见,与EBV感染无明显关系,化疗或免疫化疗效果差,预后不良。
[Abstract]:Objective lymphoproliferative disease (post-transplant lymphoproliferative disorders,PTLD) after transplantation is a serious complication after solid organ transplantation or hematopoietic stem cell transplantation. It is mainly related to iatrogenic immunosuppression and (EBV) infection of Epstein-Barr virus. Due to the low incidence, diverse clinical manifestations and pathological forms, and less prospective clinical studies, the current understanding of the disease is still very inadequate. The aim of this study was to investigate the clinicopathological features, treatment and prognosis of late-onset PTLD after renal transplantation. Methods according to the classification criteria of hematopoietic and lymphoid tissue tumors of WHO, 4 patients with renal transplantation diagnosed by PTLD, in Nanjing General Hospital of Nanjing military region from January 30 to December 30, 2001 were retrospectively analyzed for their clinicopathological data. EBV correlation examination results, treatment and prognosis, and review of relevant literature. Results all the patients were treated with triple or quadruple immunosuppressive therapy after renal transplantation. The diagnostic time from renal transplantation to PTLD was 2.518 years (median 9.5 years). One case of non-specific peripheral T-cell lymphoma (PTCL-NOS) and three cases of non-germinal center type diffuse large B-cell lymphoma (DLBCL).) EBER was negative in peripheral blood EBV-DNA and pathological tissue by in situ hybridization. 1 case of PTCL-NOS was caused by skin lesion, 3 cases by hematophagocytic histiocytosis and 3 cases by submaxillary mass, low back pain and lower extremity weakness. All patients were given immunosuppressant reduction, combined chemotherapy or rituximab combination chemotherapy. 2 of 4 patients died early, which were progression of disease and complicated with septic shock after chemotherapy. In the other two cases, after 4 months and 10 months of complete remission respectively, the disease recurred and was treated again without remission. Conclusion the late type of PTLD is mainly monomorphic type after renal transplantation, DLBCL is the most common type, and the invasion outside the knot is more common. There is no obvious relationship between late type PTLD and EBV infection. The effect of chemotherapy or immunotherapy is poor, and the prognosis is poor.
【作者单位】： 南京军区南京总医院血液科;
【分类号】：R699.2;R551.2
，
本文编号：2346502
[Abstract]:Objective lymphoproliferative disease (post-transplant lymphoproliferative disorders,PTLD) after transplantation is a serious complication after solid organ transplantation or hematopoietic stem cell transplantation. It is mainly related to iatrogenic immunosuppression and (EBV) infection of Epstein-Barr virus. Due to the low incidence, diverse clinical manifestations and pathological forms, and less prospective clinical studies, the current understanding of the disease is still very inadequate. The aim of this study was to investigate the clinicopathological features, treatment and prognosis of late-onset PTLD after renal transplantation. Methods according to the classification criteria of hematopoietic and lymphoid tissue tumors of WHO, 4 patients with renal transplantation diagnosed by PTLD, in Nanjing General Hospital of Nanjing military region from January 30 to December 30, 2001 were retrospectively analyzed for their clinicopathological data. EBV correlation examination results, treatment and prognosis, and review of relevant literature. Results all the patients were treated with triple or quadruple immunosuppressive therapy after renal transplantation. The diagnostic time from renal transplantation to PTLD was 2.518 years (median 9.5 years). One case of non-specific peripheral T-cell lymphoma (PTCL-NOS) and three cases of non-germinal center type diffuse large B-cell lymphoma (DLBCL).) EBER was negative in peripheral blood EBV-DNA and pathological tissue by in situ hybridization. 1 case of PTCL-NOS was caused by skin lesion, 3 cases by hematophagocytic histiocytosis and 3 cases by submaxillary mass, low back pain and lower extremity weakness. All patients were given immunosuppressant reduction, combined chemotherapy or rituximab combination chemotherapy. 2 of 4 patients died early, which were progression of disease and complicated with septic shock after chemotherapy. In the other two cases, after 4 months and 10 months of complete remission respectively, the disease recurred and was treated again without remission. Conclusion the late type of PTLD is mainly monomorphic type after renal transplantation, DLBCL is the most common type, and the invasion outside the knot is more common. There is no obvious relationship between late type PTLD and EBV infection. The effect of chemotherapy or immunotherapy is poor, and the prognosis is poor.
【作者单位】： 南京军区南京总医院血液科;
【分类号】：R699.2;R551.2
，
本文编号：2346502
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