University of California, Davis, School of Medicine, Department of Internal Medicine, Division of Hematology/Oncology, 4501 X Street, Suite 3016, Sacramento, CA 95817, USA.
University of California, Davis, School of Medicine, Department of Internal Medicine, Division of Rheumatology/Allergy and Clinical Immunology, 6510 GBSF, Davis, CA 95616, USA.
Autoimmun Rev. 2014 Apr-May;13(4-5):577-83. doi: 10.1016/j.autrev.2014.01.026. Epub 2014 Jan 18.
Immune thrombocytopenia, or ITP, has been recognized as a clinical entity for centuries, and the importance of humoral mechanisms in the pathophysiology of ITP has been recognized for decades. Despite the long history of the syndrome, progress in understanding its epidemiology and management has been hindered by inconsistencies in nomenclature and classification schema together with the inherent heterogeneity in characteristics of global populations and ITP-associated disorders. In the past decade, great strides have been made in devising a common language for caregivers and investigators alike through standardization definitions and outcome measures, while new tools have become available for management of its clinical manifestations. In 2009, an International Working Group presented proposed standards for definitions, classification criteria, and outcome measures. The American Society of Hematology adopted these standards in 2011, including them in that body's guideline for immune thrombocytopenia. Despite the progress made so far, 20th century interventions such as corticosteroids and IVIg remain the mainstay of therapy. However, advances in treatment have led to the introduction of targeted therapies for select patients with chronic disease. In this paper, we review aspects of the epidemiology and pathophysiology of ITP and discuss the recent changes in guidelines for nomenclature, diagnosis, and treatment.
免疫性血小板减少症（ITP）已有数百年的临床认识，而体液机制在 ITP 病理生理学中的重要性也已被认识了几十年。尽管该综合征历史悠久，但由于命名法和分类方案的不一致以及全球人群和 ITP 相关疾病的特征固有的异质性，其流行病学和管理方面的进展一直受到阻碍。在过去的十年中，通过标准化定义和结果测量，为护理人员和研究人员设计了一种通用语言，同时也为管理其临床表现提供了新的工具，从而取得了重大进展。2009 年，一个国际工作组提出了用于定义、分类标准和结果测量的建议标准。美国血液学会于 2011 年采用了这些标准，包括在该组织的免疫性血小板减少症指南中。尽管迄今为止已经取得了进展，但 20 世纪的干预措施，如皮质类固醇和 IVIg，仍然是治疗的主要方法。然而，治疗方面的进展导致针对慢性疾病患者的靶向治疗的引入。本文回顾了 ITP 的流行病学和病理生理学的各个方面，并讨论了命名法、诊断和治疗方面指南的最新变化。
