Consolini Rita, Costagliola Giorgio, Spatafora Davide
Laboratory of Immunology, Department of Clinical and Experimental Medicine, Division of Pediatrics, University of Pisa, Pisa, Italy.
Clinical Immunology and Allergy Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
Front Pediatr. 2017 Aug 21;5:179. doi: 10.3389/fped.2017.00179. eCollection 2017.
Primary immune thrombocytopenia (ITP) is the most common cause of thrombocytopenia in children and adolescents and can be considered as a paradigmatic model of autoimmune disease. This second part of our review describes the clinical presentation of ITP, the diagnostic approach and overviews the current therapeutic strategies. Interestingly, it suggests an algorithm useful for differential diagnosis, a crucial process to exclude secondary forms of immune thrombocytopenia (IT) and non-immune thrombocytopenia (non-IT), which require a different therapeutic management. Advances in understanding the pathogenesis led to new therapeutic targets, as thrombopoietin receptor agonists, whose role in treatment of ITP will be discussed in this work.
原发性免疫性血小板减少症(ITP)是儿童和青少年血小板减少症最常见的病因,可被视为自身免疫性疾病的典型模型。本综述的第二部分描述了ITP的临床表现、诊断方法,并概述了当前的治疗策略。有趣的是,它提出了一种有助于鉴别诊断的算法,这是排除继发性免疫性血小板减少症(IT)和非免疫性血小板减少症(非IT)的关键过程,而这两种情况需要不同的治疗管理。对发病机制认识的进展催生了新的治疗靶点,如血小板生成素受体激动剂,其在ITP治疗中的作用将在本文中讨论。