关键词: 血小板减少, 原发免疫性血小板减少症, 规范化治疗

Abstract:

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.

Key words: thrombocytopenia, primary immune thrombocytopenia, standardized treatment