Current Management of Primary Immune Thrombocytopenia.
Current Management of Primary Immune Thrombocytopenia.
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DOI：
10.1007/s12325-015-0251-z
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发表时间：
2015-10
影响因子：
3.8
通讯作者：
Newland AC
中科院分区：
文献类型：
--
作者：
Provan D;Newland AC
原发性免疫性血小板减少症是一种病因不明的自身免疫性疾病，影响儿童和成人。低外周血血小板计数是由于自身反应抗体过早破坏血小板以及血小板产生受损引起的。该疾病在病理生理、临床特征和治疗反应方面具有异质性。迄今为止，使用的大多数治疗方法是免疫调节药物，这些药物导致患者发病率和死亡率增加。一类新的药物，血小板生成素受体激动剂，已被开发用于ITP。这些药物已经在大量ITP患者中进行了随机对照试验。这些药物疗效高，耐受性好。此外，大约30%接受这些药物治疗的患者能够停药并维持安全或正常的血小板计数。较早的治疗方法，如脾切除术的使用比以前少，主要是因为引入了血小板生成素受体激动剂。目前正在进行评估ITP新疗法的试验，一旦试验完成，这些新疗法将在未来几年内可用。
Primary immune thrombocytopenia is an autoimmune disorder of unknown cause affecting both children and adults. The low peripheral blood platelet count is caused by premature platelet destruction by self-reacting antibodies in addition to an impairment of platelet production. The disease is heterogeneous in its pathophysiology, clinical features and responses to treatment. To date, most of the treatments used have been immune-modulating drugs and these contribute to increased morbidity and mortality in patients. A new class of drugs, the thrombopoietin receptor agonists, has been developed for use in ITP. These have gone through randomised controlled trials in large numbers of patients with ITP. These drugs have high efficacy and are well tolerated. In addition, around 30% of patients receiving these drugs are able to stop them and maintain a safe or normal platelet count. Older treatments such as splenectomy are being used less than before, largely because of the introduction of the thrombopoietin receptor agonists. Currently there are trials underway evaluating novel therapies for ITP that will become available over the next few years once the trials are complete.