规范儿童免疫性血小板减少症的诊断治疗

摘要

Immune thrombocytopenia (ITP) is a common hematological disease in clinical practice in pediatrics. ITP is classified as newly diagnosed ITP, persistent ITP, chronic ITP, and severe ITP. The criteria for assessing response to ITP treatment is complete response, response, cortieosteroid dependence, no response, and refractory ITP. The major goal for treatment of ITP is to provide a safe platelet count rather than correct the platelet count to normal levels. For patients requiring treatment, a single dose of intravenous immunoglobulin or a short course of corticosteroids can be used as first line treatment A single dose of anti-D can be used as first line treatment in Rh+,non-splenectomized children requiring treatment. The second line treatment for pediatric ITP is rituximab, anti-CD20,thrombopoietin, cyclosporin A, azathioprine, danazol, vircristine, splenectomy, and other options.%免疫性血小板减少症(immune thrombocytopenia,ITP)是儿童最常见的出血性疾病,新的分型系统将ITP分为新诊断ITP、持续性ITP、慢性ITP及重型ITP 4型,其疗效标准为完全缓解、缓解、激素依赖、无效及难治型ITP 5型.强调ITP治疗的主要目的 是提供安全的血小板计数以预防大出血,而不是将血小板提高至正常水平.推荐ITP的一线治疗为糖皮质激素、静脉注射用丙种球蛋白(IVIG)及抗D免疫球蛋白,抗CD20单抗、血小板生成素(TPO)、硫唑嘌呤、环胞素A、达那唑及长春碱类药物、脾切除等作为二线治疗.