原发免疫性血小板减少症的规范化诊治

Study on standardized diagnosis and treatment of primary immune thrombocytopenia

原发免疫性血小板减少症(primary immune thrombocytopenia,ITP)是一种获得性免疫介导的血小板减少性疾病,其发病机制为免疫失耐受导致的血小板破坏过多及巨核细胞产生血小板不足。临床表现主要为血小板减少性的出血及疲劳等症状。ITP的诊断无特异性指标,需排除其他的血小板减少性疾病。治疗目的为维持血小板在安全水平,预防出血,并提高患者生活质量。治疗指征为血小板≤30×10^(9)/L和(或)有出血表现,对于老年患者、重体力劳动者、有高血压等出血风险较高的合并症患者以及需抗血小板、抗凝治疗患者等,可适当放宽治疗指征。一线治疗为糖皮质激素及静脉注射人免疫球蛋白;二线治疗包括促血小板生成药物、利妥昔单抗及脾切除等治疗;对于难治性ITP患者,可考虑维A酸等三线治疗。

Primary immune thrombocytopenia(ITP)is an acquired immune-mediated thrombocytopenia disease,and its pathogenesis covers excessive destruction of platelets and insufficient production of platelets by megakaryocytes.Main clinical manifestations involve the bleeding related to thrombocytopenia and fatigue.No specific biomarker is found in the diagnosis of ITP,and other thrombocytopenic diseases should be excluded.The therapy is intended to maintain the safe level of platelets,prevent bleeding and improve the quality-of-life.The indication for therapy is platelet count≤30×10^(9)/L and/or active bleeding.Patients with some factors may increase the risk of bleeding:increased age,heavy physical activities,blood coagulation factor disorder,uncontrolled hypertension,and taking anticoagulants.These patients need to maintain platelet at a higher level.The first-line therapies involve corticosteroids and intravenous human immunoglobulin.The second-line therapies involve thrombopoietin receptor agonists(TPO-RAs),recombinant TPO(rhTPO),rituximab and splenectomy.All-trans retinoic acid(ATRA)and other third-line treatment should be considered for refractory ITP.