摘要
目的血管免疫母细胞性T细胞淋巴瘤(angioimmunoblatic T-cell lymphoma,AITL)是一种侵袭性淋巴瘤,本研究探讨AITL的临床病理特征、与EB病毒相关性及分子遗传学改变,并分析相关预后因素。方法选取2003-01-03-2012-12-31新疆医科大学第一附属医院有详细临床病理资料的16例AITL患者石蜡样本,EnVision法进行CXCL13、PD-1、CD10和Bcl-6免疫标记;原位杂交技术检测肿瘤EB病毒编码mRNA表达情况;荧光原位杂交技术检测肿瘤组织中Bcl-2、Bcl-6和C-MYC基因异常情况,并对所有患者进行随访。结果 16例患者,男11例(68.8%),女5例(31.2%),男女比例2.2∶1。中位年龄65.5岁。75.0%(12/16)患者国际预后指标(international prognostic indicators,IPI)评分为中高危,56.3%(9/16)患者血清乳酸脱氢酶(lactic dehydrogenase,LDH)异常,20%(2/10)患者存在骨髓侵犯,81.3%(13/16)患者临床分期为ⅢB期及以上;免疫组织化学染色结果示,CXCL13、PD-1、CD10、Bcl-6阳性率分别为93.8%(15/16,95%CI为70%~100%)、75.0%(12/16,95%CI为48%~93%)、50.0%(8/16,95%CI为25%~75%)和81.3%(13/16,95%CI为54%~96%);AITL显示特征性的CD23和CD21滤泡树突状细胞网增生,阳性率均为87.5%(14/16,95%CI为62%~98%);原位杂交:EBER阳性率为56.3%(9/16,95%CI为30%~80%);FISH检测结果示,10例AITL患者Bcl-2/IgH、Bcl-6、C-myc检测均为阴性;术后电话随访,自病理确诊之日起开始计算生存期,15例患者有随访结果。随访1~84个月,全组中位生存期为17个月;其中5例在1年内死亡,1年生存率为66.7%(5/15),总共6例患者死亡,占全组的40.0%(6/15)。单因素分析显示,年龄≥65岁、IPI评分高危组、LDH水平异常、临床病理分期ⅢB~Ⅳ期患者预后较差。结论 AITL好发于老年患者,具有高度侵袭性,预后差,CXCL13、PD-1、CD10、Bcl-6可作为一个免疫组化套餐,在该肿瘤的诊断具有重要作用,临床病理分期、IPI评分、LDH水平是影响预后的重要因素。
OBJECTIVE Angioimmunoblatic T-cell lymphoma （AITL） is an invasive lymphoma. To explore clinico- pathologic, and molecular genetic correlation with EB virus and analysis of the related prognostic factors of the AITL. METHODS Get paraffin samples of the 16 cases AITL with the detailed clinico-pathologic information were studied using immunohistochemical EnVision method for CXCL13, PD1, CD10, Bcl-6 immunolabeling. We detected the expression of EBER mRNA with in situ hybridization and the Bcl-2, Bcl-6 and C-myc gene abnormalities with interphase fluorescence in situ hybridization, and follow-up for all patients. RESULTS Sixteen cases including 11 male cases （68.8%） and 5 female cases （31. 2%）, the sex ratio was 2. 2 ： 1, the age ranged from 38--78 years old and median age was 65.5 years. AITL typically prestend with advanced stage, enlargement of the body and some lymph nodes, fever, spleno-megaly and rash, some accompanied by polyclonal hypergammaglobulinemia, 75.0% （12/16） were high-risk/high-risk with International Prognosis Index （IPI）, 56.3% （9/16） presented abnormalities with lactate dehydrogenase （LDH）, 20%（2/10） patients had bone marrow involvement, 81.3% （13/i6） patients with clinical stage HI B period and above. Immunophenotype analysis showed neoplastic cells expressed the following markers： CXCL13 （positive rate 93. 8%,15/16）, PD-1 （positive rate75.0%, 12/16）, CD10 （positive rate 50. 0%, 8/16）, Bcl-6 （positive rate 81. 3%, 13/16）, CD21 （positive rate 87.5%,14/16）, CD23 （positive rate 87.5%,14/16）, EBER （positive rate 56.3%,9/16）. The genetic alterations of Bcl-2, Bcl-6 and C-MYC was not found using FISH technique, respectively. Postoperative follow-up with telephone, starting from the date of the pathological diagnosis of survival, 15 patients had Xollow-up results, follow- up of 1--84 months, the median survival time was 17 months （1-84 months）;5 cases died within a year, one year survival rate was 66.7 % （5/15）, All data was used for statistical analysis. 6 cases died （mortality rate 40.0 %, 6/15）. Univa- riate analysis showed that., the age ≥65 years, IPI score risk group, LDH level exception and clinical stage roB--IV, the prognosis of patients was poor, all statistical data analysis. CONCLUSIONS The AITL is generally more common in eld- erly patients, with an advanced stage and a poor prognosis;CXCL13, PD-1, CD10, Bcl-6 plays an important role in the diagnosis of the tumor. Clinical staging, IPI score and LDH levels are important prognostic factors.
出处
《中华肿瘤防治杂志》
CAS
北大核心
2017年第6期368-373,共6页
Chinese Journal of Cancer Prevention and Treatment
