血管免疫母细胞性T细胞淋巴瘤12例临床分析

Angioimmunoblastic T cell lymphoma: Clinical analysis of 12 cases

目的研究血管免疫母细胞性T细胞淋巴瘤(AITL)的临床、病理特征及预后。方法回顾性分析经病理证实的12例AITL患者的临床特点、病理形态及免疫表型、治疗和生存情况。结果12例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。确诊依据淋巴结活检,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,免疫表型全部为成熟外周T细胞性。12例均用CVP为主的方案化疗,总有效率58%。3年生存率为25%,全组中位生存20个月。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方案。

Objective To study the clinicopathological characteristics and prognosis of patients with angioimmunoblastic T cell lyphoma （AITL）. Methods Clinicopathological features, immunophenotypes, treatment and survival of 12 AITL patients with their diagnosis confirmed by pathology were retrospectively analyzed. Results The main symptom of the 12 patients was general lymphadenopathy, 9 of them had fever. AITL, diagnosed based on lymph-node biopsy, was characterized by damage to normal structure of lymphonodus, proliferation of immunoblastic cells, and arborescent supervascularization. Immunophenotypes were observed in mature peripheral T-cell lymphoma. CVP regimen was the most commonly used chemotherapy. Fifty-eight percent of patients had a good initial response to chemotherapy. The 3-year survival rate was 25%, with a median survival time of 20 months. Conclusion The clinical course of most AITL cases is aggressive. The disease may progress rapidly with a poor prognosis. Further study is required to improve the outcome.