成人系统性间变大细胞淋巴瘤临床分析

Clinical analysis of adult systemic anaplastic large-cell lymphoma

目的探讨成人系统性间变大细胞淋巴瘤(sALCL)患者的临床特征及预后。方法回顾性分析110例成人sALCL患者的临床资料。根据免疫组化结果将患者分为间变淋巴瘤激酶(ALK)阳性(+)组76例和ALK阴性(-)组34例。患者均采用含蒽环类药物的化疗或辅以放疗,按照非霍奇金淋巴瘤国际疗效判断标准进行疗效评价。比较两组患者的临床特征、总有效率、完全缓解率、复发率,并分析两组患者的生存率和预后因素。结果两组中位发病年龄、AnnArbor分期、血清乳酸脱氢酶(LDH)水平差异均有统计学意义(均P<0.05),而两组性别、血红蛋白、白蛋白、有无B症状、体能状态、国际预后指数评分、有无结外侵犯器官、侵犯部位差异均无统计学意义(均P>0.05)。两组的总有效率、完全缓解率、复发率差异均无统计学意义(均P>0.05)。ALK(-)组5年无进展生存期(PFS)率、5年总生存率低于ALK(+)组(P<0.05)。LDH升高、体能状态≥2分、不含依托泊苷化疗方案为ALK(+)组5年PFS的独立不良预后因素。体能状态≥2分为ALK(-)组5年PFS的独立不良预后因素。结论不同ALK表型的sALCL患者因ALK表型不同而有不同的临床特征;ALK(+)患者5年总生存率高于ALK(-)患者,LDH升高、体能状态≥2分、不含依托泊苷化疗方案为ALK(+)患者5年PFS的独立不良预后因素。体能状态≥2分为ALK(-)患者5年PFS的独立不良预后因素。

Objective To investigate the clinical characteristics and prognosis of adult systemic anaplastic large-cell lymphoma(sALCL). Methods The clinical data of 110 adult patients with sALCL were analyzed retrospectively. According to the results of immunohistochemistry,the patients were divided into anaplastic lymphoma kinase(ALK)-positive group( n =76) and ALK-negative group( n =34).All patients were treated with anthracycline-containing chemotherapy or adjuvant radiotherapy,and the efficacy was evaluated according to the international efficacy evaluation criteria for non-Hodgkin′s lymphoma.Clinical characteristics,total effective rate,complete remission rate,and recurrence rate were compared between the two groups,and survival rate and prognostic factors were analyzed in the two groups. Results Median age of onset,Ann Arbor stage,and serum lactate dehydrogenase(LDH) level were significantly different between the two groups(all P <0.05),furthermore,there were no statistically significant differences in gender, hemoglobin ,albumin,B symptoms,performance status,international prognostic index score,extranodal invasion of organs,or invasion sites between the two groups(all P >0.05). No statistically significant difference was found in total effective rate,complete remission rate,or recurrence rate between the two groups(all P >0.05).The ALK-negative group had lower 5-year progression-free survival(PFS) rate and 5-year overall survival rate than the ALK-positive group( P <0.05).Elevated LDH,performance status≥2 points,and etoposide-free chemotherapy regimen were independent adverse prognostic factors for 5-year PFS in the ALK-positive group.Performance status ≥2 points was an independent adverse prognostic factor for 5-year PFS in the ALK-negative group. Conclusion sALCL patients with different ALK phenotypes represent different clinical characteristics;ALK-positive patients have a higher 5-year overall survival rate than ALK-negative patients, elevated LDH,performance status ≥2 points,and etoposide-free chemotherapy regimen are independent adverse prognostic factors for 5-year PFS in ALK-positive patients.Performance status ≥2 points is an independent adverse prognostic factor for 5-year PFS in ALK-negative patients.