血管免疫母细胞型T细胞淋巴瘤的临床特点及预后分析

Clinical features and prognostic factors of angioimmunoblastic T cell lymphoma

目的 探讨血管免疫母细胞型T细胞淋巴瘤（AITL）的临床特点及预后影响因素.方法 收集18例有完整治疗及随访记录的AITL患者的临床和随访资料,总结其临床特征,并进行生存分析.患者初治时均接受标准的CHOP样方案治疗,4例曾进行放疗,1例接受了巩固性的大剂量化疗联合自体造血干细胞移植.对6例患者外周血中的T细胞、B细胞和自然杀伤（NK）细胞亚群进行了流式细胞检测.结果 18例患者中位年龄为55岁,男女比例2.6：1.Ⅲ～Ⅳ期和有B症状者占72.2%,贫血者占47.1%,69.2%的患者免疫球蛋白增高,60.0%的患者乳酸脱氢酶（LDH）增高.初治完全缓解（CR）8例,疾病进展（PD）10例,CR率为44.4%.2例多程化疗后的患者,应用沙利度胺治疗,1例获得部分缓解（PR）,1例CR,无疾病进展生存时间分别为2个月和6＋个月.2年总生存率和2年无病生存率分别为62.2%和44.4%.单因素分析结果 显示,发病年龄和原发耐药情况与生存期有关,发病年龄≥30岁、Ann Arbor分期晚、有B症状和脾肿大与无病生存期缩短有关.4例患者在治疗中出现重症肺炎,其中2例死于呼吸衰竭.6例进行了外周血淋巴细胞比例的流式细胞检测的患者中,5例CD3＋ CD4＋ T细胞、自然杀伤细胞和B细胞比例降低,而CD3＋ CD8＋ T细胞比例升高.结论 AITL患者存在天然免疫和获得性免疫功能的下降,一线应用CHOP样方案治疗的疗效欠佳,沙利度胺用于AITL的治疗值得深入研究,年龄〈30岁和初治敏感的AITL患者预后较好.

Objective To retrospectively analyze the clinical features and prognostic factors of patients with angioimmunoblastic T-cell lymphoma （AITL）. Methods The clinicopathological and follow-up data of 18 AITL patients undergoing integrated treatment from Feb. 1998 to April 2009 in our department were retrospectively analyzed.All of the patients received CHOP-like regimens as initial chemotherapy, including 4 once treated with radiotherapy and 1 with high dose therapy followed by autologous stem cell transplantation （ HDT-ASCT） as upfront consolidation therapy. B-cell, T-cell and NK-cell subgroup proportions in the peripheral blood were tested by flow cytometry in 6 patients. Results The median age of the 18 patients was 55 years, male and female ratio was 2.6：1. Seventy-two percent of the patients were in an advanced stage. 72% of them had B symptoms, 69% hypergammaglobulinemia, 60% elevated LDH and 47% anemia.Forty-four percent achieved CR after initial treatment with CHOP-like regimens. With the median follow-up of 26 months, the overall 2-year survival and disease free survival （ DFS） rates were 62.2% and 44.4% , respectively. In the univariate analysis, only age 〉30 years and primary refractory disease adversely affected overall survival （ OS）; age 〉 30 years, advanced stage,B symptoms and splenomegaly adversely affected DFS. Four patients suffered from severe pneumonia during treatment, 2 of them died of respiratory failure. Flow cytometry of peripheral blood lymphocytes showed that 5 of the 6 tested cases had decreasing proportion of CD3＋ CD4＋ T cells, B cells and NK cells but elevated CD3＋ CDS＋ T cells. Two heavily treated patients achieved partial and complete response by thalidomide therapy, with a progression free survival （PFS） of 2 and 6 ＋ months, respectively. Conclusion AITL patients do not response well to CHOP-like regimens chemotherapy. Age 〈30 years and sensitive to initial chemotherapy are associated with prolonged OS. Effectiveness of thalidomide in the treatment of AITL deserves further investigation. Peripheral blood lymphocytes test indicates that AITL patients suffered from both natural and acquired immune defects.