原发系统型间变性大细胞淋巴瘤的临床特点及预后分析  

AnaIysis of clinical characteristics and prognostic factors for systemic anaplastic large cell lymphoma

作　　者：熊竹娟[1] 周祥[1] 吴萍[1] 魏雯[1] 敬小梅[1] 任苑蓉[1] 李力[1] 周进[1] 张智慧[1] 

机构地区：[1]四川省肿瘤医院淋巴瘤病区,成都610041

出　　处：《国际输血及血液学杂志》2015年第4期277-282,共6页International Journal of Blood Transfusion and Hematology

基　　金：四川省卫生厅基金资助项目（120039）

摘　　要：目的探讨原发系统型间变大细胞淋巴瘤（ALCL）的临床特征及预后。方法选择2010年1月至2015年1月四川省肿瘤医院淋巴瘤病区收治的21例初治原发系统型ALCL患者作为研究对象。比较间变淋巴瘤激酶（ALK）阳性ALCL患者与阴性患者经环磷酰胺＋表柔比星＋长春新碱＋泼尼松（cHOP）类方案治疗后的总有效率（0RR）及总生存（0s）率的差异。单因素分析患者年龄、性别、美国东部肿瘤协作组（ECOG）评分、Ann-Arbor分期、国际预后指数（IPI）评分、B症状、乳酸脱氢酶（LDH）水平等对预后的影响。结果本组21例患者中，ECOG评分O～1分为18例（85．7％），≥2分为3例（14．3％）；AnnArbor分期Ⅰ～Ⅱ期为5例（23．8％），nI～Ⅳ期为16例（76．2％）；IPI评分0～1分为9例（42．9％），2～3分为12例（57．1％）。所有患者均有淋巴结肿大（100．0％），其中8例合并结外受侵（38．1％）；伴有B症状为10例（47．6％）；伴乳酸脱氢酶（LDH）水平升高为10例（47．6％）。ALK^＋。ALCL患者为13例（61．9％），ALK-ALCL为8例（38．1％），二者各项临床资料比较，差异均无统计学意义（P〉0．05）。本组患者接受CHOP类方案化疗后，ORR为71．4％。ALK^＋ALCL患者ORR为92．3％；ALK-ALCL患者ORR为37．5％；二者ORR及OS率比较，差异均有统计学意义（P=0．14；x^2=4．064，P=0．044）。单因素分析结果显示，ALK-和LDH水平升高是预后不良的影响因素，而性别、年龄、ECOG评分、AnnArbor分期、IPI评分、结外受侵、B症状对患者的ORR及OS率影响，差异均无统计学意义（P〉0．05）。结论原发系统型ALCL诊断时分期晚，B症状及结外受侵常见；ALK^＋ALCL患者接受CHOP类方案化疗后，ORR和OS率高，ALK^-和LDH水平升高是原发系统型ALCL预后不良的影响因素。Objective To study clinical characteristics and prognostic factors in systemic anaplastic large cell lymphoma（ALCL）. Methods From January 9,010 to January 2015, 21 systemic ALCL patients who were admitted in Department of Lymphoma, Siehuan Cancer Hospital were included in this study. All this patients were treated with cyclophosphamide, epirubicin, vincristine, and prednisone （CHOP） like chemotherapy. The overall response ra＇te （ORR） and overall survival （OS） rate of anaplastic lymphoma kinase （ALK） positive patients were compared with that of ALK- patients. Age, sex, Eastern Cooperative Oncology Group （ECOG） score, Ann-Arbor stage, B symptoms, international prognostic index （IPI） score, lactate dehydrogenase （LDH） level, extranodal invasion were analyzed for prognosis by univariate analysis. Results In these 21 cases patients, 18 cases （85.7%） had 0-1 ECOG score, 16 cases （76.2%） were in ]]I-IV Ann Arbor stage, and 12 cases （57. 1%） had IPI score over 2. All of these patients had lymphadenectasis （100.0%）. Eight cases （38.1%） had extranodal disease at diagnosis, 10 cases （47.6 %） had B symptoms, and 10 cases （47.6%） had elevated LDH level. Thirteen cases （61.9%） were ALK＋ patients, and 8 cases （38.1%） were ALK-. There were no significant differences of clinical data between ALK^＋ and ALK^- patients （P〉0.05）. ORR of ALCL patients with CHOP like chemotherapy was 71.4%. ORR of ALK＋ patients was 92. 3%, and that of ALK- patients was 37. 5%. There were differences of ORR and OS rate between ALK＋ and ALK- patients （P〈0.014 ; x^2= 4. 064, P = 0. 044）. ALK expressionand LDH level were important prognostic factors which proved by univariate analysis results （P〈0.05）, but gender, age, ECOG score, Ann Arbor stage, IPI score, extranodal invasion and B symptoms were not affect significant on ORR and OS rate of ALCL patients （P〈0. 05）. Conclusions Most patients with systemic ALCL usually have late stage, B symptoms and extran

关 键 词：淋巴瘤 淋巴瘤 大细胞 间变性 预后 间变性淋巴瘤激酶 

分 类 号：R733.1[医药卫生—肿瘤]