原发于纵膈的T淋巴母细胞淋巴瘤临床特征及预后因素分析(英文)  

Analysis of Clinical Characteristics and Prognostic Factors of Primary Mediastinal T-cell Lymphoblastic Lymphoma

作　　者：晋鑫[1] 赵海丰[1] 于泳[1] 赵丹丹[3] 杨洪亮[1] 王晓芳[1] 赵智刚[1] 王亚非[1] 吴晓雄[2] 达万明[3] 张翼鷟[1] 

机构地区：[1]天津医科大学附属肿瘤医院血液科,天津市肿瘤防治重点实验室,天津300060 [2]解放军总医院第一附属医院血液科,北京100037 [3]解放军总医院血液科,北京100853

出　　处：《中国实验血液学杂志》2013年第2期377-382,共6页Journal of Experimental Hematology

基　　金：supported by grants from the National Natural Science Foundation of China(30672208 and 81100337)

摘　　要：淋巴母细胞淋巴瘤占成人非霍奇金淋巴瘤的2%-4%,其中85%-90%为T细胞型。本研究旨在分析原发纵膈的T淋巴母细胞淋巴瘤患者的相关特点及预后,以期提高对该病的认识。通过对1998年1月至2011年1月确诊为原发纵膈的T淋巴母细胞淋巴瘤35例病例的分析,对疾病的临床特点及相关预后因素进行了总结。结果表明,35例病例中男性24例,女性11例,平均年龄19岁(年龄区间:5-52岁),多数病人临床分期为Ⅲ/Ⅳ期,16例(45.7%)患者表现为纵膈巨大肿块,浆膜腔积液(胸腔、心包腔)也很常见(62.9%),3年总生存率及3年无进展生存率分别为36%和24%,5年总生存率及5年无进展生存率则分别为25%和16.7%。统计分析表明,贫血是影响患者总生存率的独立预后因素(P=0.048),巨大肿块(P=0.048),上腔静脉综合征(P=0.021)及确诊时血小板异常(P=0.021)为影响患者无进展生存率的独立预后因素。结论:原发纵膈的T淋巴母细胞淋巴瘤发病率低、预后差、生存期短,患者确诊时多为晚期,伴贫血和巨大肿块及上腔静脉综合征等症状的患者预后更差。Lymphoblastic lymphoma （LBL） comprises 2% to 4% of non-Hodgkin lymphomas cases in adults, of which 85% to 90% of LBL in adults is of T-ceU phenotype. This study was aimed to evaluate the clinical characteristics and prognostic factors of patients with mediastinal T-LBL. Based on the retrospective analysis of the clinical data of 35 patients with mediastinal T-LBL during the period from January 1998 to January 2011, the clinical characteristics and prognostic factors of mediastinal T-LBL were summarized. The results showed that the total of 35 patients were identified （male 24 and female 11）, with a median age of 19（5 -52） years. The majority of patients were in stage Ⅲ/Ⅳ, 16 cases （45.7%） presented bulky mediastinal mass. Intrathoracic effusions （pleural, pericardial） were not uncommon （62.9%）. Overall survival rate （OS） and progression - free survival rate （PFS） at 3 years for the entire cohort were 36% and 24%, respectively. OS and PFS at 5 years were 25% and 16.7%, respectively. Anemia at diagnosis were an important, independent predictor of OS （ P = 0. 048 ）. Bulky mass （ P = 0. 048 ）, superior vena cava syndrome （ P = 0. 021 ）, and abnormal PLT count at diagnosis was the independent prognostic factors for PFS （ P = 0. 021 ）. It is concluded that the patients with primary mediastinal T-LBL are characterized by a low incidence, bad prognosis, and short survival. For patients accompanying with anemia, bulky mass and superior vena cava syndrome, their prognosis is worse.

关 键 词：纵膈 T淋巴母细胞淋巴瘤 预后因素 贫血 

分 类 号：R733.1[医药卫生—肿瘤]