【正文】 response to ITP treatments ? CR: platelet count ≥ 100 109/L and absence of bleeding ? R: platelet count ≥ 30 109/L and at least 2fold increase the baseline count and absence of bleeding ? Time to response: time from starting treatment to time of achievement of CR or R ? NR: platelet count 30 109/L or less than 2fold increase of baseline platelet count or bleeding ? Loss of CR or R: platelet count below 100 109/L or bleeding (from CR) or below 30 109/L or less than 2fold increase of baseline platelet count or bleeding (from R) 治療反應的界定 ? Corticosteroiddependence ? The need for ongoing or repeated doses administration of corticosteroids for at least 2 months to maintain a platelet count at or above 30 109/L and/or to avoid bleeding (patients with corticosteroid dependence are considered nonresponders) 治療反應的界定 ? For response criteria in refractory ITP ? HRQoL indicates healthrelated qualityoflife assessment. ? Platelet counts should be confirmed on at least 2 separate occasions (at least 7 days apart when used to define CR, R) or 1 day apart when used to define NR or loss of response. ? Baseline platelet count refers to platelet count at the time of starting of the investigated treatment。 ? 致命性出血常發(fā)生在血小板低于 30 109/L成年 ITP患者。 原發(fā)性、繼發(fā)性 ITP（ primary secondary immune thrombocytopenia） 的定義 ? 除一些罕見繼發(fā)性 ITP（如輸血相關紫癜）保留原名外，其他統(tǒng)稱為“繼發(fā)性血小板減少癥”（ secondary ITP ），并用括號注明基礎疾病。 自身反應性 T細胞在 ITP的發(fā)病機制中起重要作用。最小量的皮質激素是慢性 ITP的治療原則。最佳方案尚待確定 , 目前常用方案為 375 mg/(m2 停藥后血小板計數(shù)逐漸下降 , 高于30 109 /L 的持續(xù)時間為 1 ～ 2周。在 118例成人慢性難治性 ITP患者中進行的隨機對照試驗表明 , Eltrombopag 3 個劑量組(每日 50和 75 mg) 的有效率 (血小板計數(shù) ≥50 109 /L) 分別為 28%、 70%和 81% , 而安慰劑組僅為 11%。常見不良事件為頭痛 ( 31% ) 、乏力 ( 24% ) 、腹瀉 ( 24% ) 、鼻衄 ( 23% ) 、鼻咽炎 ( 21% ) 和關節(jié)痛 ( 20% ) , 發(fā)生率與劑量無明顯關系。此外 , 自身抗體可介導巨核細胞損傷 , 降低巨核細胞對 TPO 的反應性。 難治性 ITP定義、治療目標及 反應評估 ? Definition (all should be met) ? Failure to achieve at least R or loss of R after splenectomy ? Need of treatment(s) (including, but not limited to, low dose of corticosteroids) to minimize the risk of clinically significant bleeding. Need of ondemand or adjunctive therapy alone does not qualify the patient as refractory. ? Primary ITP confirmed by excluding other supervened causes of thrombocytopenia 難治性 ITP定義、治療目標及 反應評估 ? Definition of ondemand therapy ? Any therapy used to temporarily increase the platelet count sufficiently to safely perform invasive procedures or