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目的探讨血管免疫母细胞性T细胞淋巴瘤(AITL)的临床病理特征及治疗。方法分析1997年2月至2004年7月收治的14例AITL。结果14例就诊的AITL患者主要症状为全身淋巴结肿大,9例伴有发热等全身症状,3例并发自身免疫性溶血性贫血。病理组织学呈淋巴结结构破坏,免疫母细胞增生,树枝状血管增生的特点,免疫表型全部为T细胞性。14例均用ProMACE-CytaBOM方案化疗,CR3例,PR5例,总有效率57%。2年生存率为60%,全组中位生存25个月,3例缓解的患者已无瘤生存超过5年。结论AITL临床过程呈侵袭性,进展快,中位生存期短,预后差,应探索更为有效的治疗策略。
Objective To investigate the clinicopathological features and treatment of angioimmunoblastic T cell lymphoma (AITL). Methods A total of 14 patients with AITL admitted from February 1997 to July 2004 were analyzed. Results The main symptoms of 14 AITL patients were generalized lymphadenopathy, 9 cases with general symptoms of fever and 3 cases with autoimmune hemolytic anemia. Histopathology showed the destruction of lymph node structure, immunoblastic proliferation, dendritic vascular proliferation characteristics, all of the immune phenotype T cell. All 14 patients were treated with ProMACE-CytaBOM regimen. The CR3 and PR5 patients had a total effective rate of 57%. The 2-year survival rate was 60%, with a median survival of 25 months in the entire group and 3 patients who had remission survived without disease for more than 5 years. Conclusion The clinical course of AITL is aggressive, with rapid progression, short median survival and poor prognosis. A more effective treatment strategy should be explored.