McMillan Robert, Nugent Diane
The Scripps Research Institute, La Jolla, California 92037, USA.
Int J Hematol. 2005 Feb;81(2):94-9. doi: 10.1532/ijh97.04168.
Immune thrombocytopenic purpura (ITP) is a disorder manifested by isolated thrombocytopenia. In vivo infusion studies in the 1950s and 1960s provided evidence that the thrombocytopenia was due to autoantibody-induced platelet destruction. However, there is mounting evidence that platelet production in this disorder may also be suppressed by antibodies. Early morphologic studies showed megakaryocytic damage in ITP, and these results have been confirmed by ultrastructural studies. Autologous platelet turnover studies in the 1980s showed that most ITP patients have either normal or reduced platelet turnover rather than increased turnover, as would be expected if platelet destruction were the only pathogenetic mechanism. More recently, in vitro culture studies of both adult and pediatric ITP have shown that some ITP plasmas suppress both megakaryocytopoiesis and thrombopoiesis. In view of these findings, both platelet destruction and suppression of platelet production seem likely to be involved in the pathogenesis of ITP.
免疫性血小板减少性紫癜(ITP)是一种以孤立性血小板减少为特征的疾病。20世纪50年代和60年代的体内输注研究提供了证据,表明血小板减少是由自身抗体诱导的血小板破坏所致。然而,越来越多的证据表明,该疾病中的血小板生成也可能受到抗体的抑制。早期的形态学研究显示ITP中巨核细胞受损,这些结果已通过超微结构研究得到证实。20世纪80年代的自体血小板周转率研究表明,大多数ITP患者的血小板周转率正常或降低,而非如血小板破坏是唯一致病机制时所预期的那样升高。最近,成人和儿童ITP的体外培养研究均显示,一些ITP血浆会抑制巨核细胞生成和血小板生成。鉴于这些发现,血小板破坏和血小板生成抑制似乎都参与了ITP的发病机制。