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目的 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 ,其确诊靠淋巴结活检 ,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。
Objective To analyze the clinical manifestations of AITL in 6 children and provide basis for the diagnosis and treatment of children with AITL. Methods The clinical data of 6 AITL inpatients admitted in the recent 10 years were retrospectively analyzed. Results The clinical manifestations of AITL were characterized by fever, rash, hepatosplenomegaly, anemia and hypergammaglobulinemia. The pathological morphology was characterized by the so-called triad symptom. The immunophenotype T and B lymphocytes were all expressed in T Mainly. Conclusion The disease has a high degree of malignancy, its diagnosis by lymph node biopsy, prognosis larger individual differences between the prognosis and adult worth exploring, the treatment should be based on the disease selected individualized treatment.