18例血管免疫母T细胞性淋巴瘤临床病理特征与诊断

目的：探讨血管免疫母T细胞淋巴瘤（angioimmunoblastic T-cell lymphoma, AITL)的临床特征和病理表现，为本病的诊断提供依据。方法：回顾分析2002年7月至2007年9月中南大学湘雅医院明确诊断的18例AITL患者的临床和病理资料，其中男9例，女9例，发病年龄从14~70岁。结果：AITL主要表现为淋巴结肿大、发热、脾大和皮疹，伴有多克隆性高球蛋白血症和其他血液学异常如白细胞升高、Coombs试验阳性，常累及骨髓，具有特征性的病理组织学表现，免疫组织化学实验显示B-淋巴细胞趋化因子CXCL13阳性率为93.3%。结论：反复多次淋巴结等部位的活检有助于本病确诊，病理组织学检查结合免疫组织化学分型如CXCL13等具有重要的诊断和鉴别诊断意义。

Clinicopathologic features and diagnosis of 18 patients with angioimmunoblastic T-cell lymphoma

ObjectiveTo explore the clinical and pathologic features of angioimmunoblastic T-cell lymphoma(AITL) and provide evidence for diagnosis.MethodsEighteen AITL patients (9 males and 9 females aged from 14 to 70 years) were retrospectively analyzed in Xiangya Hospital of Central South University from July 2002 to September 2007. ResultsCharacteristic features at the presentation of AITL included generalized lymphadenopathy, fever, splenomegaly, and skin rashes with polyclonal hyper-gammaglobulinemia and other hematological abnormalities (such as Coombs-positive hemolytic anemia), which often involved the bone marrow and had well-described histologic features.The positive rate for CXCL13 was 93.3%. ConclusionRepeated lymphadenbiopsy is helpful for AITL diagnosis. Routine histological and immunohistochemical examinations (especially including CXCL13) play significant role in the diagnosis and differential diagnosis of AITL.