儿童难治性特发性血小板减少性紫癜的治疗进展

Advancement in treatment of childhood refractory idiopathic thrombocytopenic purpura

特发性血小板减少性紫癜(ITP)是一种自身免疫性疾病,是临床最常见的出血性疾病。治疗以糖皮质激素、免疫抑制剂、脾切除等非特异性手段为主,但副反应明显,且约1/3的患儿治疗无效,成为难治性ITP。儿童难治性ITP目前尚无特效根治药物及方法,治疗应个体化,治疗选择应根据血小板计数和出血状态而定。近年对ITP自身免疫发病机制的深入研究令许多新的定向免疫干预措施开始进入临床研究阶段,实施定向免疫干预将是今后ITP诊疗的方向。文章结合ITP的发病机制对这些进展作一概述。

Idiopathic thrombocytopenic purpura （ITP） is the most common autolmmune and hemorrhagic disease. Non-specificity methods such as glucocorticoid, immune depressant and splenectomy are main treatments, but the adverse effects are obvious. Treatment is ineffective in one thirds of ITP children, who will transform to refractory ITP. While no effective therapy for these patients at the present, treatment should be individualized and based on the condition of platelet count and situation of hemorrhage. The lucubrate to the immunopathogenesis in ITP leads many original directional immunologic interventions come to the stage of clinical research. The direction of diagnosis and treatment for ITP is to enforce directional immunologic intervention. Progresses of treatment and pathogenesis in childhood chronic refractory ITP are summarized.