系统型间变性大细胞淋巴瘤30例临床分析

 目的　探讨系统型间变性大细胞淋巴瘤（S-ALCL）的临床特征和预后相关因素。方法　回顾性分析30例S-ALCL患者的临床资料。30例患者均以联合化疗为主，配合局部病灶野放疗8例。化疗方案主要为 CHOP、EPOCH、Hyper-CVAD，以CHOP方案为主。结果　30例S-ALCL患者中位年龄36岁，男女比例为1.5∶1，有B症状、Ⅲ～Ⅳ期和结外侵犯者分别占60.0 %（18／30）、73.3 %（22／30）和60.0 %（18／30）；乳酸脱氢酶（LDH）升高者占46.7 %（14／30）；间变性大细胞淋巴瘤激酶（ALK）+ 18例（60.0 %），其发病年龄小于ALK- 者（u＝3.92，P＝0.001）。单因素分析显示ALK-及LDH升高是重要的预后不良因素。结论　S-ALCL患者发病年龄较轻，预后较好。但ALK-、LDH升高者预后不良。治疗以联合化疗为主，对于有不良预后因素的患者，大剂量治疗可能获益。

Analysis of clinical characteristics of 30 cases with systemic anaplastic large cell lymphoma

Objective To study clinical characteristics and prognostic factors in systemic anaplastic large cell lymphoma （S-ALCL）. Methods Clinical date of 30 systemic ALCL patients from Department Hemotology of Shanxi Cancer Hospital from December 1998 to September 2009 were reviewed. Results The median age of the 30 patients was 36 years old. The male/female ratio was 1.5：1.18 （60.0 %） 18/30 of the patients had B symptoms, 22 （73.3 %） had Ann Arbor stage Ⅲ-Ⅳ, and 18 （60.0 %） had extranodal disease at diagnosis. 14 （46.7 %） had elevated lactate dehydrogenase （LDH） level. The positive rate of ALK was 18 （60.0 %）. Compared with ALK negative patients, ALK-positive patients were younger （u = 3.92, P =0.001）. ALK expression and LDH level were important prognostic factors by Long-rank test. Conclusion S-ALCL usually occur in younger patients, with good response to chemotherapy and good prognosis. ALK negative and elevated LDH level are correlated to unfavorable prognosis. Combination chemotherapy is the major choice to these patients. High-dose chemotherapy maybe benefit to the patients who have adverse prognostic factors.