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腎移植后晚發(fā)型淋巴組織增生性疾病的臨床病理特點(diǎn)

發(fā)布時(shí)間:2018-11-21 09:00
【摘要】:目的移植后淋巴組織增生性疾病(post-transplant lymphoproliferative disorders,PTLD)是實(shí)體器官移植或造血干細(xì)胞移植后的一種嚴(yán)重并發(fā)癥,主要與醫(yī)源性免疫抑制和Epstein-Barr病毒(EBV)感染有關(guān)。由于發(fā)生率低,臨床表現(xiàn)和病理形態(tài)多樣,前瞻性臨床研究少,目前對(duì)該病的認(rèn)識(shí)還很不充分。本研究旨在探討腎移植后晚發(fā)型PTLD的臨床病理特點(diǎn)及治療和預(yù)后。方法 2001-01-30-2013-12-30在南京軍區(qū)南京總醫(yī)院根據(jù)WHO造血與淋巴組織腫瘤分類標(biāo)準(zhǔn)共確診4例腎移植后PTLD,回顧性分析該組患者的臨床病理資料、EBV相關(guān)檢查結(jié)果及治療和預(yù)后,并對(duì)相關(guān)文獻(xiàn)進(jìn)行復(fù)習(xí)。結(jié)果 4例患者腎移植后均采用三聯(lián)或四聯(lián)免疫抑制治療,腎移植至PTLD診斷時(shí)間為2.5~18年(中位9.5年)。4例的病理類型均為單形型,其中1例非特指型外周T細(xì)胞淋巴瘤(PTCL-NOS)和3例非生發(fā)中心型彌漫大B細(xì)胞淋巴瘤(DLBCL)。外周血EBV-DNA和病理組織原位雜交檢測(cè)EBER均陰性。1例PTCL-NOS以皮膚病變起病,合并噬血細(xì)胞淋巴組織細(xì)胞增生癥,3例DLBCL分別以頜下腫塊、腰背痛和下肢無力起病。確診后均予免疫抑制劑減量,同時(shí)行聯(lián)合化療或利妥昔單抗聯(lián)合化療。4例患者中,2例早期死亡,分別為疾病進(jìn)展和化療后并發(fā)感染性休克。另2例分別在完全緩解4個(gè)月和10個(gè)月后疾病復(fù)發(fā),再次治療未緩解。結(jié)論腎移植后晚發(fā)型PTLD以單形型為主,DLBCL最常見,結(jié)外侵犯多見,與EBV感染無明顯關(guān)系,化療或免疫化療效果差,預(yù)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.
【作者單位】: 南京軍區(qū)南京總醫(yī)院血液科;
【分類號(hào)】:R699.2;R551.2
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本文編號(hào):2346502

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