儿童血管免疫母细胞性T细胞淋巴瘤临床分析

通过对6例儿童血管免疫母细胞性T细胞淋巴瘤(AITL)临床表现的分析,为儿童AITL的诊断和治疗提供依据。方法回顾性分析近10年收治的6例AITL住院患者的各项临床资料。结果AITL临床表现以发热、皮疹、肝脾淋巴结肿大。贫血和高丙种球蛋白血症为特点,病理学形态表现为所谓的三联征,免疫表型T、B淋巴细胞均有表达,以T为主。结论该病具有较高的恶性程度,其确诊靠淋巴结活栓,预后个体差异较大,其预后与成人的差异性值得探讨,治疗上宜根据病情选用个体化的治疗方法。

Clinical Analysis of Angioimmunoblastic T-cell Lymphoma in Children

Objective To improve the diagnosis and therapy of children angioimmunoblatic T-cell lymphoma (AITL) by analyzing the clinical parameters of AITL. Methods The clinical data of 6 patients with AITL in the recent 10 years were retrospectively analyzed. Results The main symptoms of AITL had fever,skin rash,general lymphadenopathy, hepatosplenomegaly, anemia and polyclonal hypergammaglobulinemia. The pahologic morphology of AITL was what was known as "Triad", the immunophenotype of both the T-cell and B-cell was positive, and B-cell's one was main. Conclusion AITL is high malignant, and only lymphaden biopsy could confirm the diagnosis of AITL. Its prognosis is variable in different patients. The discrepancy of prognosis between children and adults is worthy of study, and individualized therapy of AITL must be adopted.