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Refractory primary immune thrombocytopenia: treatment options

机译:难治性原发性免疫性血小板减少症:治疗选择

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The primary immune thrombocytopenia (PIT) is an acquired autoimmune disorder characterized by transient or persistent decreased platelet count with increased risk of bleeding. The chronic form of this disease primarily affects adults. Since spontaneous remissions are rare, patients usually are treated with steroids from the start. Approximately one third of the cases does not respond to steroid regimens; the second-line treatment is splenectomy, achieving the highest cure rates. However, between 10 to 30% of patients do not respond to spleen surgical removal, which is the group of patients with refractory chronic PIT. There is no evidence on whether this therapeutic regimen is most effective in these cases, so the treatment remains empirical. In this paper, various treatment options that can be used in refractory chronic PIT, the action mechanism of drugs, doses and more frequent adverse effects were reviewed.
机译:原发性免疫性血小板减少症(PIT)是一种获得性自身免疫性疾病,其特征是血小板计数暂时或持续减少,出血风险增加。这种疾病的慢性形式主要影响成年人。由于自发缓解很少见,因此通常从一开始就使用类固醇治疗患者。大约三分之一的病例对类固醇疗法无反应;二线治疗是脾切除术,可达到最高治愈率。但是,有10%至30%的患者对脾脏手术切除无反应,这是顽固性慢性PIT患者。没有证据表明这种治疗方案在这些情况下是否最有效,因此治疗仍是经验性的。本文综述了可用于难治性慢性PIT的各种治疗选择,药物的作用机理,剂量和更常见的不良反应。

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