Qin Yan, Shi Yuan-Kai, He Xiao-Hui, Yang Jian-Liang, Zhang Chang-Gong, Zhou Sheng-Yu, Liu Xin-Fan, Liu Peng, Yang Sheng, Zhou Li-Qiang, Han Xiao-Hong, Yao Jia-Rui
Department of Medical Oncology, Cancer Institute & Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijng 100021, China.
Zhonghua Zhong Liu Za Zhi. 2010 Jun;32(6):448-51.
To retrospectively analyze the clinical features and prognostic factors of patients with angioimmunoblastic T-cell lymphoma (AITL).
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.
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(+)CD8(+) 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.
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.
回顾性分析血管免疫母细胞性T细胞淋巴瘤（AITL）患者的临床特征及预后因素。
回顾性分析1998年2月至2009年4月在我科接受综合治疗的18例AITL患者的临床病理及随访资料。所有患者均接受CHOP样方案作为初始化疗，其中4例接受过放疗，1例接受高剂量治疗后行自体干细胞移植（HDT-ASCT）作为 upfront 巩固治疗。6例患者采用流式细胞术检测外周血B细胞、T细胞和NK细胞亚群比例。
18例患者的中位年龄为55岁，男女比例为2.6:1。72%的患者处于晚期。72%的患者有B症状，69%有高球蛋白血症，60%乳酸脱氢酶升高，47%有贫血。44%的患者在初始接受CHOP样方案治疗后达到完全缓解（CR）。中位随访26个月，总体2年生存率和无病生存率（DFS）分别为62.2%和44.4%。单因素分析显示，仅年龄>30岁和原发难治性疾病对总生存（OS）有不利影响；年龄>30岁、晚期、B症状和脾肿大对DFS有不利影响。4例患者在治疗期间发生严重肺炎，其中2例死于呼吸衰竭。外周血淋巴细胞流式细胞术检测显示，6例检测病例中有5例CD3(+)CD4(+)T细胞、B细胞和NK细胞比例降低，但CD3(+)CD8(+)T细胞升高。2例接受过大量治疗的患者通过沙利度胺治疗获得部分缓解和完全缓解，无进展生存期（PFS）分别为2个月和6+个月。
AITL患者对CHOP样方案化疗反应不佳。年龄<30岁且对初始化疗敏感与延长OS相关。沙利度胺治疗AITL的有效性值得进一步研究。外周血淋巴细胞检测表明AITL患者存在天然和获得性免疫缺陷。
