Objective: To analyze the clinical features and prognostic factors of primary systemic anaplastic large cell lymphoma (ALCL) . Methods: 40 ALCL cases treated in the First Affiliated Hospital of Zhejiang University from January 2013 to December 2018 were retrospectively analyzed. Results: ① With a median age of 41 (14-67) years, there were 29 males and 11 females, 36 patients (90.0%) had Ann Arbor stage Ⅲ-Ⅳ tumors, 23 patients (57.5%) were in high-intermediate or high international prognostic index (IPI) risk group. 25 patients (62.5%) had B symptoms, such as fever, emaciation and night sweat.38 patients (95.0%) had extranodal invasion, 25 patients (62.5%) had higher LDH level, and 25 patients (62.5%) had high expression of Ki-67 (80% or more) . With 22 ALK(+) patients (55.0%) and 18 ALK(-) patients (45.0%) , there was a significantly difference in the median age of the two groups [29 (14-67) years old vs 51.5 (19-67) years old, P=0.003]. ② All patients received chemotherapy, 18 cases were treated with CHOP (cyclophosphamide, doxorubicin, vindesine, prednisone) , 12 cases with ECHOP (cyclophosphamide, doxorubicin, vindesine, prednisone, etoposide) , 10 cases with other treatments and 26 patients (65.0%) obtained complete remission (CR) . ALK(-) (P=0.029, OR=13.458) and Ki-67 expression of 80% or more (P=0.04, OR=14.453) were independent factors of CR rate, the CR rate of ECHOP chemotherapy was higher than CHOP chemotherapy (P=0.026) . ③ LDH level, IPI score, ALK expression and chemotherapy regimen had significantly effect on progression free survival (PFS) and overall survival (OS) (P<0.05) . Conclusion: The study shows that primary systemic ALCL usually occurs in males, the average age of ALK(+) patients were younger than ALK(-) patients. Most patients are in stage Ⅲ-Ⅳ with extranodal invasion, more than half of the patients have B symptoms, elevated LDH, and high expression of Ki-67. The expression level of Ki-67, ALK expression, and chemotherapy regimen have prognostic value for CR rate, the LDH level, IPI score, ALK expression and chemotherapy regimen for PFS and OS. ECHOP is a better choice with improved prognosis.
目的： 分析原发系统型间变性大细胞淋巴瘤（ALCL）的临床特征及预后因素。 方法： 回顾性分析2013年1月至2018年12月期间在浙江大学医学院附属第一医院住院治疗的40例ALCL患者的临床资料，总结其临床特征并分析其预后相关因素。 结果： ①40例患者中男29例，女11例，中位年龄41（14~67）岁，Ann Arbor分期Ⅲ~Ⅳ期36例（90.0%），IPI评分中高危、高危23例（57.5%）；25例（62.5%）有B症状，发热最为常见，其次为消瘦、盗汗；38例（95.0%）有结外侵犯；25例（62.5%）LDH升高；25例（62.5%）Ki-67≥80%；ALK阳性22例（55.0%），中位年龄29（14~67）岁，ALK阴性18例（45.0%），中位年龄51.5（19~67）岁，年龄差异有统计学意义（P=0.003）。②40例患者均接受化疗，采用CHOP（环磷酰胺+多柔比星+长春地辛+泼尼松）方案18例，ECHOP（依托泊苷+CHOP）方案12例，其他方案10例；26例（65.0%）获得完全缓解（CR），ALK阴性（P=0.029，OR=13.458）和Ki-67≥80%（P=0.04，OR=14.453）是CR的独立影响因素，ECHOP方案的CR率高于CHOP方案[83.3%（10/12）对38.9%（7/18），P=0.026]。③LDH水平、IPI评分、ALK表达情况以及化疗方案的选择对无进展生存（PFS）和总生存（OS）影响有统计学意义（P<0.05）。 结论： ALCL发病男性居多，青少年至中老年均有发病，ALK阳性中年轻患者居多，诊断时临床分期晚，多数患者有结外侵犯，半数以上患者有B症状、LDH升高、Ki-67高表达；ALK表达与否、Ki-67表达水平以及化疗方案的选择可影响疗效，LDH水平、IPI评分、ALK表达与否以及化疗方案的选择可影响PFS和OS；ECHOP是较优的化疗方案，可改善预后。.
Keywords: Anaplastic large cell lymphoma; Anaplastic lymphoma kinase; Clinical features; Prognostic factors.