Objective: To evaluate the clinical characteristics, treatment and prognosis of systemic anaplastic large cell lymphoma(sALCL).
Methods: The clinical data of 90 cases with sALCL treated in the Department of Hematology of the Affiliated Xijing Hospital of Air Force Medical University from November 2018 to October 2021 were retrospectively analyzed. The clinical features, treatment and prognosis were summarized and the prognostic factors were investigated.
Results: There were 58 males and 32 females, with a median age of 32 (12-73) years old. 69 (76.7%) patients had Ann Arbor stage Ⅲ-Ⅳ disease and half of the patients had extranodal infiltration. The median age was 27(12-72) years of the 60 ALK+ patients while 53(15-73) years of the 30 ALK- patients, and it was significantly different in the age of onset between the two group(P<0.01). 88 patients received first line chemotherapy, and 50(568%) cases achieved complete remission(CR). IPI score≥3 was an independent risk factor for CR. The median progressive free survival(PFS) and overall survival(OS) of the patients were not reached. Multivariate analysis showed that no achievement of CR after first-line therapy was a significant prognostic factor influencing PFS and OS.
Conclusion: sALCL mainly occurs in males and most patients were in advanced stage. Half of the patients had extranodal involvement. The CR rate after first-line chemotherapy was 568%, and IPI score≥3 was a significant prognostic factor for CR. No achievement of CR after first-line therapy is poorly prognostic for PFS and OS.
题目: 系统性间变大细胞淋巴瘤患者的临床特点及预后分析.
目的: 评价系统性间变大细胞淋巴瘤（sALCL）患者的临床特征、治疗及预后.
方法: 回顾性分析2018年11月至2021年10月空军军医大学西京医院血液内科收治的90例sALCL患者的临床资料，总结其临床特点、治疗及预后，并探讨预后影响因素.
结果: 90例sALCL患者中男性58例，女性32例，中位年龄32（12－73）岁。69例（76.7%）患者发病时Ann Arbor 分期为Ⅲ－Ⅳ期，半数患者有结外病变。ALK+患者60例，中位年龄27（12-72）岁，ALK-患者30例，中位年龄53（15-73）岁，两组的发病年龄有显著统计学差异（P<0.001）。88例患者接受标准一线化疗，50例（568%）患者达完全缓解（CR）。IPI评分≥3分是影响一线化疗后CR的独立危险因素。本组患者中位无进展生存（PFS）及中位总生存（OS）未达到。多因素分析结果显示，一线化疗后未达CR是PFS及OS的独立不良预后因素.
结论: sALCL以男性居多，诊断时多处于进展期，50%患者合并结外病变。一线化疗后CR率为56.8%，IPI≥3分是一线化疗后CR的不良影响因素。一线化疗后未达CR是PFS及OS 的不良预后因素.
Keywords: anaplastic large cell lymphoma; clinical characteristics; prognosis.