经HTLV-1前病毒基因检测确诊的12例成人T细胞白血病/淋巴瘤临床特征及预后分析  

Clinical characteristics and prognosis in 12 patients with adult T cell leukemia/lymphoma confirmed by HTLV- 1 provirus gene detection

作　　者：吴正军[1] 郑晓云[1] 杨小珠[1] 刘庭波[1] 杨婷[1] 郑志宏[1] 高飞[1] 陈成璇[1] 李景岗[1] 张臣青[1] 林武强 郑合勇 林素霞[3] 胡建达[1] 

机构地区：[1]福建医科大学附属协和医院,福建省血液病学重点实验室,福建省血液病研究所,福州350001 [2]莆田市第一医院血液科 [3]莆田学院附属医院血液科

出　　处：《中华血液学杂志》2016年第12期1027-1032,共6页Chinese Journal of Hematology

基　　金：国家临床重点专科建设项目[闽卫医函（2011）1018号];福建省2012年临床重点专科建设项目[闽卫科教（2012）149号]

摘　　要：目的 探讨成人T细胞白血病/淋巴瘤（ATLL）患者的临床特征及预后.方法 对2013年3月至2015年7月23例诊治疑似ATLL患者的外周血标本,采用PCR法和测序技术检测其基因组DNA中的l型人类T细胞白血病病毒（HTLV-1）前病毒DNA,阳性患者确诊ATLL;分析确诊患者的临床特征,并随访其生存情况.结果 23例疑似患者中12例确诊为ATLL,男女比例为2∶1,中位年龄51（28～66）岁,均来自福建沿海HTLV-1小流行区.12例患者中除l例为淋巴瘤型外,余均为急性白血病型.临床特征中,ATLL细胞检出率高[81.8％（9/11）,典型6例,不典型3例],肝、脾、淋巴结肿大多见,高钙血症、LDH升高常见.ATLL细胞典型免疫表型以CD4^＋CD8^-为主.合并HBV感染率高达54.5％.10例接受化疗,其中2例获完全缓解后接受异基因造血干细胞移植.截至随访结束日期,7例死亡,4例存活,l例失访,中位生存期2.8（0.9～10.8）个月.HTLV-1前病毒基因监测提示,l例患者在移植后HTLV-1前病毒基因转阴.结论 在福建省沿海地区ATLL并不罕见,ATLL具有典型的临床特征,预后不佳.Objective To analyze the clinical characteristics and prognosis of adult T cell leukemia/lymphoma （ATLL）.Methods Peripheral blood samples from patients who were suspected as ATLL from March,2013 to July,2015,were collected for HTLV-1 provirus genes detection in genomic DNA extraction by PCR.Cases showing positive results were confirmed as ATLL.Clinical and laboratory characteristics,therapeutic outcomes and survival evaluation were collected.Results 12 out of 23 suspected patients were confirmedly diagnosed as ATLL through HTLV-1 provirus genes detection by PCR.Eight patients were male and four patients were female.Median age was 51 （range 28-66） years old.All of those patients came from coastal cities of Fujian province where a HTLV-1 epidemic area locates.In the subtype classification of these 12 ATLL,11 patients were classified as acute type and one case as lymphoma type ATLL.As one of the clinical characteristics of ATLL,＇flower cells＇,with typical or atypical morphology had been observed in a high rate （81.8%）.Clinical symptom such as hepatomegaly,splenomegaly and lymphadenectasis were detected in most of patients,and hypercalcemia and elevated LDH were also noted commonly.The ATLL cells immunophenotype were typical,and the major subtype was CD4＋CD8-type.Confection of hepatitis B virus was detected in a high rate （54.5%）.Ten patients received chemotherapy,and 2 cases in complete remission after chemotherapy received allogeneic hematopoietic stem cell transplantation.At the end of the follow-up,7 cases died,4 cases survived,1 case was lost,and the median survival was 2.8 （0.9-10.8） months.We found a case had HTLV-1 provirus negative after transplantation.Conclusion In the coastal area of Fujian Province,ATLL is not rare.Characteristics of those ATLL are typical.But prognosis is still unsatisfactory.

关 键 词：人类嗜T淋巴细胞病毒1型 白血病淋巴瘤 成人T细胞 高钙血症 乙型肝炎病毒 

分 类 号：R733[医药卫生—肿瘤]