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成人免疫性血小板缺乏症之傳統療法及處置

Traditional and New Approaches to the Management of Adult Immune Thrombocytopenia

摘要


成人免疫性血小板缺乏紫斑症(Immune Thrombocytopenic Purpura, ITP)被認為是一種自體免疫性疾病,疾病主要特徵是血小板數目過低及黏膜出血。成人多發生在四十歲以下的年輕女性,病程多為慢性且需要治療;成人死亡率小於百分之五,發病機轉至今仍未明瞭,但多數認為是體內產生抗體來對抗自己的血小板,而造成血小板加速破壞所致。近來許多研究對疾病的病理學有更深的探討,因此發現新的作用機轉藥物可用來治療ITP,本文將簡介傳統治療藥物及新作用機轉藥物臨床試驗結果,提供脾臟切除治療外更多種選擇。

並列摘要


Immune thrombocytopenic purpura is an autoimmune disorder characterized by a low platelet count and mucocutaneous bleeding. It is defined as a peripheral blood platelet count of less than 100×109/L, and the absence of any obvious initiating and/or underlying cause. The disease in adults is generally chronic, the onset is often insidious, and recently epidemiological data suggests that the incidence in adults is approximately equal for the sexes except in the mid-adult years (30-60 years), when the disease is more prevalent in women. Bleeding after previous surgery, dentistry, and trauma should be considered when estimating the possible duration of chronic thrombocytopenia or an alternative bleeding disorder. The mechanisms of thrombocytopenia in ITP have shifted from the traditional view of increased platelet destruction mediated by auto-antibodies to more complex mechanisms in which both impaired platelet production and T cell-mediated effects play a role. This document aims to report on new data and provide consensus-based recommendations relating to diagnosis and treatment of ITP in adults, while providing a wide approach to treatment. However, treatment related decisions still remain principally dependent on clinical expertise or patient preference, rather than high-quality clinical trial evidence.

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