Gernsheimer Terry
Division of Hematology, University of Washington School of Medicine, Seattle, WA 98104, USA.
Eur J Haematol Suppl. 2008 Feb(69):3-8. doi: 10.1111/j.1600-0609.2007.00998.x.
Immune thrombocytopenic purpura (ITP) is characterized by the presence of antiplatelet antibodies and immune platelet destruction. The disorder has been described as having a predilection for young women over men. Bone marrow megakaryocytes appear morphologically and quantitatively normal, and early platelet kinetic studies were consistent with reduced platelet survival as the primary abnormality in ITP. During the last 10-20 yr, understanding of the kinetics of this disorder has evolved with evidence that platelet survival is not as abbreviated as previously thought. Thrombopoietin levels are only minimally elevated, if at all, suggesting marrow stimulation and platelet production may not be maximized. Megakaryocyte physiology appears to be altered in ITP, also suggestive of diminished platelet production. It appears both platelet survival and production are impaired in ITP. The epidemiology of ITP is reviewed here and the pathophysiology of ITP is reconsidered.
免疫性血小板减少性紫癜(ITP)的特征是存在抗血小板抗体和免疫性血小板破坏。该疾病被描述为女性多于男性。骨髓巨核细胞在形态和数量上看起来正常,早期血小板动力学研究表明血小板存活时间缩短是ITP的主要异常。在过去10 - 20年中,随着有证据表明血小板存活时间并不像以前认为的那样缩短,对该疾病动力学的认识有所发展。血小板生成素水平即使有升高也只是轻微升高,这表明骨髓刺激和血小板生成可能未达到最大化。巨核细胞生理学在ITP中似乎发生了改变,这也提示血小板生成减少。看来ITP中血小板存活和生成均受损。本文对ITP的流行病学进行了综述,并重新审视了ITP的病理生理学。