成人慢性免疫性血小板减少性紫癜的治疗进展

Therapeutic advance of adult chronic immune thrombocytopenic purpura

成人慢性免疫性血小板减少性紫癜 (ITP)自身抗原 (GPⅡb/Ⅲa、GPⅠb Ⅸ )的修饰或分解以及激活的T细胞表达CD4 0L为抗体产生的重要步骤 ,除PAIgG之外 ,RP %和TPO测定是ITP有用的辅助诊断指标。血小板 >5 0× 10 9/L、无出血的患者一般无需治疗。治疗上主要采取非特异性免疫抑制或封阻巨噬细胞的Fc受体。抗 D治疗可延缓或免除脾切除术 ,但长期应用耗费可观。对切脾无反应的患者 (血小板 <30× 10 9/L ,有活动性出血 )最常推荐的治疗为IVIg、糖皮质激素、副脾切除和其他适当的药物 (达那唑、硫唑嘌呤、长春新碱类及环磷酰胺等 )。

Modified or processed autoantigen (GPⅡb/Ⅲa、GPⅠb Ⅸ) and expressing CD40L of activated T cells were important procedures of antibody production in adult chronic immunologic thrombocytopenic purpura (ITP). Except platelet associated IgG (PAIgG), determination of the percentage of reticulated platelet (RP%) and plasma thrombopoietin level (TPO) were useful assistant markers. Treatments were not necessary for the ITP patients with platelet >50×10 9 /L and no bleeding symptoms. Nonspecific immunosuppression or blocking Fc receptors of macrophages were employed at treatment of chronic ITP. Anti D immunoglobulin treatment could postpone and spare splenectomy, but if used in individual patients for long term therapy (>1 year),the cost of anti D could become significant enough to be prohibitive. The following options for the management of chronic ITP in patients who fail to respond ( platelets < 30×10 9 /L,with active bleeding) to splenectomy are recommended: IVIg, glucocorticosteroid, accessory splenectomy and other appropriate drugs (danazol, azathioprine, vinca alkaloids and cyclophosphamide and so on).