Kuwana Masataka
Institute for Advanced Medical Research, Keio University School of Medicine.
Nihon Rinsho. 2003 Apr;61(4):670-5.
Idiopathic thrombocytopenic purpura(ITP) is an autoimmune disease characterized by increased platelet destruction caused by anti-platelet autoantibodies, which mainly target a platelet surface antigen, GP IIb-IIIa. CD4+ T cells reactive with GP IIb-IIIa play a primary role in the disease process, since these autoreactive T cells are involved in the production of pathogenic anti-platelet autoantibodies. GP IIb-IIIa-reactive T cells mainly recognize 'cryptic' determinants on the amino-terminal extracellular domains of both GP IIb alpha and GP IIIa, and are activated primarily in the spleen. These findings are particularly useful for clarifying the pathogenic process in ITP patients and for developing a therapeutic approach that blocks pathogenic anti-platelet antibody production.
特发性血小板减少性紫癜(ITP)是一种自身免疫性疾病,其特征是抗血小板自身抗体导致血小板破坏增加,这些抗体主要靶向血小板表面抗原GP IIb-IIIa。与GP IIb-IIIa反应的CD4+ T细胞在疾病过程中起主要作用,因为这些自身反应性T细胞参与了致病性抗血小板自身抗体的产生。与GP IIb-IIIa反应的T细胞主要识别GP IIbα和GP IIIa氨基末端细胞外结构域上的“隐蔽”决定簇,并且主要在脾脏中被激活。这些发现对于阐明ITP患者的致病过程以及开发阻断致病性抗血小板抗体产生的治疗方法特别有用。