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摘要: 目的 分析间变性淋巴瘤激酶(ALK)阳性间变性大细胞淋巴瘤(ALCL)的临床特征，探讨治疗效果、预后影响因素及临床治疗方案。方法 收集2014年12月—2021年12月在徐州医科大学附属医院初治的12例ALK+ ALCL患者的临床资料，回顾性分析其临床特点、治疗效果及预后生存情况。结果 12例患者的中位发病年龄为28(14~45)岁，其中男11例。11例(91.67%)患者初诊时评估晚期(Ⅲ~Ⅳ期)，7例(58.33%)患者合并B症状，5例(41.67%)患者LDH升高，4例(33.33%)患者以浅表淋巴结肿大为首发部位，7例(58.33%)患者伴有腹腔淋巴结肿大。6例(50.00%)患者伴有结外侵犯，3例(25%)患者≥2个结外部位侵犯。2例(16.67%)患者IPI评分为高中危/高危组(3~5分)；7例(58.33%)患者aaIPI评分为高中危/高危组(3~5分)。Ki-67≥80%、结外侵犯≥2处、aaIPI 3~5分、一线化疗方案是影响初始化疗后达CR的不良因素。CRP升高、结外侵犯≥2处、IPI 3~5分、aaIPI 3~5分、Ki-67≥80%、一线化疗方案是影响患者PFS的预后因素，IPI评分是影响OS的预后因素。3例患者联合自体造血干细胞移植后始终处于临床缓解状态。结论 ALK+ ALCL多见于男性，发病年龄较早，大多数患者诊断时处于疾病晚期。一线化疗的选择，对ALK+ ALCL患者初始CR率以及PFS均有影响；自体造血干细胞移植作为巩固疗效或复发患者选择的治疗方案疗效可观。
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关键词:
- ALK阳性间变性大细胞淋巴瘤 /
- 临床特征 /
- 预后因素
Abstract: Objective To analyze the clinical characteristics of ALK-positive anaplastic large cell lymphoma(ALCL), and to explore the treatment effect, prognostic factors and clinical treatment plan.Methods The clinical data of 12 ALK+ ALCL patients who were initially treated in the Affiliated Hospital of Xuzhou Medical University from December 2014 to December 2021 were collected. The clinical characteristics, therapeutic effect and prognosis were retrospectively analyzed.Results The median age of the patients was 28 years(14-45 years), including 11 males. Eleven patients(91.67%) had advanced stage(Ⅲ-Ⅳ) at initial diagnosis, 7 patients(58.33%) had B symptoms, 5 patients(41.67%) had elevated LDH, 4 patients(33.33%) had superficial lymphadenopathy as the initial site, and 7 patients(58.33%) had abdominal lymphadenopathy. Six patients(50.00%) had extranodal invasion, and 3 patients(25.00%) had ≥2 extranodal invasion. According to IPI score, 2 patients (16.67%) were in intermediate-high risk/high risk group (3-5 points); According to aaIPI score, 7 patients (58.33%) were in intermediate-high risk/high risk group (3-5 points). Ki-67≥80%, extranodal invasion≥2, aaIPI 3-5, and first-line chemotherapy regimen were the adverse factors affecting CR after initial chemotherapy. Elevated CRP, extranodal invasion≥2, IPI score 3-5, aaIPI score 3-5, Ki-67≥80%, and first-line chemotherapy regimen are the prognostic factors for PFS, and IPI score was the prognostic factor for OS. Three patients remained in clinical remission after autologous hematopoietic stem cell transplantation.Conclusion ALK+ ALCL is more common in men, the age of onset is young, and most patients are in the late stage of disease when diagnosed. The choice of first-line chemotherapy has an impact on the initial CR rate and PFS of ALK+ ALCL patients. ASCT is effective as the treatment of choice for consolidation or patients with recurrence. -
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表 1 12例患者临床特征
患者序列 性别 年龄/岁 CRP LDH B症状 结外侵犯≥2处 分期 初始治疗后达CR 生存状态 1 男 45 升高 正常 是 是 Ⅳ 否 生存 2 男 35 正常 正常 否 否 I 是 生存 3 男 24 正常 正常 否 否 Ⅳ 是 生存 4 男 28 正常 升高 是 否 Ⅳ 是 生存 5 男 38 正常 正常 否 否 Ⅲ 是 生存 6 男 26 正常 正常 是 否 Ⅲ 否 生存 7 男 38 升高 正常 是 否 Ⅳ 否 死亡 8 男 36 升高 升高 否 是 Ⅳ 否 生存 9 女 15 正常 升高 是 是 Ⅲ 否 生存 10 男 42 升高 升高 否 否 Ⅲ 是 生存 11 男 19 正常 正常 是 否 Ⅳ 是 生存 12 男 14 正常 升高 是 否 Ⅲ 是 生存 
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表 2 影响12例患者初始化疗CR的单因素分析
例(%) 临床因素 例数 初始治疗后达CR P 临床因素 例数 初始治疗后达CR P 年龄/岁 1.000 结外侵犯≥2处 0.045 ≤30 6 4(66.7) 否 9 7(77.8) >30 6 3(50.0) 是 3 0 B症状 0.293 Ki-67/% 0.015 是 7 3(42.9) <80 6 6(100.0) 否 5 4(80.0) ≥80 6 1(16.7) 血沉/(mm/h) 0.576 IPI评分 0.152 <50 8 4(50.0) 低危/低中危组 10 7(70.0) ≥50 4 3(75.0) 高中危/高危组 2 0 CRP 0.072 aaIPI评分 0.028 正常 7 6(85.7) 低危/低中危组 5 5(100.0) 升高 5 1(20.0) 高中危/高危组 7 2(28.6) LDH 0.558 治疗含VP16 0.028 正常 7 5(71.4) 否 7 2(28.6) 升高 5 2(40.0) 是 5 5(100.0)
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