间变大细胞淋巴瘤临床特点及预后分析

目的：探讨间变大细胞淋巴瘤(anaplastic large cell lymphoma，ALCL)的临床病理特点及预后。方法：回顾性分析22例ALCL患者的临床资料，并评价不同治疗方案的疗效。结果：22例患者的中位发病年龄为32(9~70)岁，中位病程2个月，间变性淋巴瘤激酶(anaplastic lymphoma kinase，ALK)阳性者占68.2%(15/22)。22例患者均进行了化学治疗，初始化学治疗采用CHOP(环磷酰胺、阿霉素、长春新碱、泼尼松)、CHOPE(CHOP联合依托泊苷)或BEACOP(CHOP联合依托泊苷+博莱霉素)方案，14例(63.6%)患者达完全缓解，ALK阳性患者的初治完全缓解率显著高于ALK阴性患者(P<0.05)，年龄、性别、分期、β2-微球蛋白(β2 microglobulin，β2-MG)水平、乳酸脱氢酶(lactatedehydrogenase，LDH)水平、B症状对完全缓解率的影响均无统计学意义(P>0.05)。中位随访时间41(2~150)个月，12例存活，中位无进展时间为22.5(2~150)个月，年龄、性别、分期、国际预后指数、ALK表达、β2-MG水平、LDH水平、B症状对生存的影响均无统计学意义(P>0.05)。结论：ALCL以ALK阳性为主，化学治疗仍是主要治疗方法，CHOPE方案是较好的初治方案，多数患者预后较好。

Clinical features and prognosis for anaplastic large cell lymphoma

Objective: To determine clinical and pathologic profi les for anaplastic large cell lymphoma (ALCL).Methods: Th e clinical data of 22 patients with ALCL were analyzed retrospectively. Th erapentieeff ect of diff erent treatment strategies on ALCL was evaluated.Results: Th e median age for these patients was 32(9–70) years old and the patients with positiveALK accounted for 68.2% (15/22). All patients underwent chemotherapy, including regimentsof CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone), CHOPE (CHOP plusetoposide) or BEACOP (CHOP plus etoposide and bleomycin). Fourteen (63.6%) patientsachieved initial complete remission (CR) and the CR rate for patients with ALK+ was signifi cantlyhigher than that of patients with ALK– (P<0.05), while the age, gender, stage, beta 2-microglobulin(2-MG) level, lactate dehydrogenase (LDH) level, B symptoms had no significant effect on the rateof CR (P>0.05). After a median follow-up of 41 (2–150) months, 12 patients were overall survival,the median progression free time was 22.5 (2–150) months, and the age, gender, stage, IPI index,ALK expression level, beta 2-MG level, LDH level, and B symptoms had no significant effect on therate of overall survival (P>0.05).Conclusion: ALK-positive occurs mainly in ALCL patients. The chemotherapy is still the maintreatment, and CHOPE regimen is a better initial treatment scheme because the most patients showgood prognosis.