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目的:回顾性分析本院单中心近年收治的血管免疫母细胞性T细胞淋巴瘤(AITL)的临床特点及常规化疗近期疗效。方法:1999年9月～2010年9月于本院明确诊断AITL患者23例,其中21例接受治疗。初次治疗17例予CHOP样或CHOP方案,4例予左旋门冬酰胺酶+博莱霉素+地塞米松+长春地辛方案;化疗后予受累野照射每组各1例,予自体造血干细胞移植巩固治疗每组各1例。复发后予ICE、DHAP或ProMACE/CytaBOM方案化疗。维持治疗采用干扰素或联合沙利度胺治疗者5例,采用西达苯胺者3例。结果:发病中位年龄60岁,男:女为1.9:1,Ann ArborⅢ～Ⅳ期占96%,57%患者有B组症状,22%患者合并脾受侵/脾肿大。实验室检查结果示,39%患者乳酸脱氢酶升高,75%患者β2微球蛋白升高,80%患者D-二聚体升高。病理组织免疫组化结果显示,CXCL13阳性率100%(12/12),EBER阳性率80%(8/10)疗效结果分析,CHOP样方案组7例获CR,8例获PR;含左旋门冬酰胺酶方案组1例获CR,1例获PR。21例患者中位生存27(2.9～51.1)个月,3年、5年总生存率分别为44%、29%。化疗后90%患者出现Ⅲ～Ⅳ度骨髓抑制,33%患者出现肺部感染,1例患者发生带状疱疹。结论:AITL为具有独特临床病理和生物学行为的外周T细胞肿瘤,多数患者同时存在凝血机制异常。AITL患者因并发免疫功能异常,化疗后骨髓抑制及感染问题不容忽视。
Objective: To retrospectively analyze the clinical features of AITL treated in recent years and the recent curative effects of conventional chemotherapy. Methods: From September 1999 to September 2010, 23 patients with AITL were diagnosed in our hospital, of which 21 were treated. The first treatment of 17 cases given CHOP-like or CHOP program, 4 cases of levascal amidase + bleomycin + dexamethasone + vindesine program; after chemotherapy to the affected field irradiation in each group of 1 case of autologous hematopoietic stem cells Transplant consolidation therapy in each group of 1 case. After recurrence to ICE, DHAP or ProMACE / CytaBOM regimen chemotherapy. Maintenance therapy with interferon or combined thalidomide in 5 patients, the use of catecholam in 3 cases. Results: The median age at onset was 60 years, with a male to female ratio of 1.9 to 1 and Ann Arbor stage III to IV accounting for 96%. 57% of patients had symptoms of group B. 22% had splenomegaly / splenomegaly. Laboratory tests showed elevated lactate dehydrogenase in 39% of patients, elevated β2 microglobulin in 75%, and elevated D-dimer in 80% of patients. Immunohistochemical results of pathological tissue showed that the positive rate of CXCL13 was 100% (12/12), and the positive rate of EBER was 80% (8/10). The results of CHOP-like group were 7 cases of CR and 8 cases of PR; One case of asparaginase group received CR, and one case received PR. The median survival time was 27 (2.9-5.1) months in 21 patients. The 3-year and 5-year overall survival rates were 44% and 29%, respectively. Ninety percent of patients after chemotherapy had grade III-IV myelosuppression, 33% had pulmonary infection, and 1 had herpes zoster. Conclusion: AITL is a peripheral T cell tumor with unique clinicopathological and biological behaviors. Most patients have abnormal coagulation mechanism. AITL patients due to concurrent immune dysfunction, bone marrow suppression and infection after chemotherapy can not be ignored.