Università di Modena e Reggio Emilia, Modena, Italy.
J Clin Oncol. 2013 Jan 10;31(2):240-6. doi: 10.1200/JCO.2011.37.3647. Epub 2012 Aug 6.
The International Peripheral T-Cell Lymphoma Project was undertaken to better understand the subtypes of T-cell and natural killer (NK) -cell lymphomas.
Angioimmunoblastic T-cell lymphoma (AITL) was diagnosed according to the 2001 WHO criteria by a central review process consisting of panels of expert hematopathologists. Clinical, pathologic, immunophenotyping, treatment, and survival data were correlated.
Of 1,314 patients, 243 (18.5%) were diagnosed with AITL. At presentation, generalized lymphadenopathy was noted in 76% of patients, and 89% had stages III to IV disease. Skin rash was observed in 21% of patients. Hemolytic anemia and hypergammoglobulinemia occurred in 13% and 30% of patients, respectively. Five-year overall and failure-free survivals were 33% and 18%, respectively. At presentation, prognostic models were evaluated, including the standard International Prognostic Index, which comprised the following factors: age ≥ 60 years, stages III to IV disease, lactic dehydrogenase (LDH) > normal, extranodal sites (ENSs) > one, and performance status (PS) ≥ 2; the Prognostic Index for Peripheral T-Cell Lymphoma, comprising: age ≥ 60 years, PS ≥ 2, LDH > normal, and bone marrow involvement; and the alternative Prognostic Index for AITL (PIAI), comprising: age > 60 years, PS ≥ 2, ENSs > one, B symptoms, and platelet count < 150 × 10(9)/L. The simplified PIAI had a low-risk group (zero to one factors), with 5-year survival of 44%, and a high-risk group (two to five factors), with 5-year survival of 24% (P = .0065).
AITL is a rare clinicopathologic entity characterized by an aggressive course and dismal outcome with current therapies.
国际外周 T 细胞淋巴瘤项目旨在更好地了解 T 细胞和自然杀伤（NK）细胞淋巴瘤的亚型。
血管免疫母细胞性 T 细胞淋巴瘤（AITL）的诊断依据是 2001 年世卫组织标准，通过由专家血液病理学家组成的专家组进行中心审查。对临床、病理、免疫表型、治疗和生存数据进行了相关性分析。
在 1314 名患者中，有 243 名（18.5%）被诊断为 AITL。就诊时，76%的患者出现全身淋巴结肿大，89%的患者为 III 至 IV 期疾病。21%的患者出现皮疹。溶血性贫血和高丙种球蛋白血症分别发生在 13%和 30%的患者中。5 年总生存率和无失败生存率分别为 33%和 18%。就诊时，评估了预后模型，包括标准的国际预后指数，包括以下因素：年龄≥60 岁、III 至 IV 期疾病、乳酸脱氢酶（LDH）>正常、结外部位（ENSs）>1 个、以及体能状态（PS）≥2；外周 T 细胞淋巴瘤的预后指数，包括：年龄≥60 岁、PS≥2、LDH>正常和骨髓受累；以及替代的 AITL 预后指数（PIAI），包括：年龄>60 岁、PS≥2、ENSs>1 个、B 症状和血小板计数<150×10^9/L。简化的 PIAI 具有低危组（零至一个因素），5 年生存率为 44%，高危组（两个至五个因素），5 年生存率为 24%（P=0.0065）。
AITL 是一种罕见的临床病理实体，具有侵袭性病程和目前治疗方法下的不良预后。
