成人原发免疫性血小板减少症诊治中国专家共识(2012修订版)解读.ppt

Estimated annual rate of bleeding according to age group ITP 出血评分量表 因 素 ? 分 值 年 龄 瘀 点/瘀 斑 器 官 出 血 ≥65岁 头面部 其它部位 鼻衄/牙龈出血/口腔血疱 内脏出血（肺、胃肠道、泌尿生殖系统） 中枢系统 偶发、可自止 多发、持续不止 不伴贫血 伴有贫血 危及生命 ? 1 √ ? √ ? ? ? ? ? ? 2 ? √ ? √ ? ? ? ? ? 3 ? ? ? ? √ √ ? ? ? 4 ? ? ? ? ? ? √ ? ? 5 ? ? ? ? ? ? ? √ √ 王琳 侯明.原发免疫性血小板减少症出血评分系统临床应用分析。 中华血液学杂志。2012 发病机制 对自身抗原免疫耐受缺失 血小板生成减少 血小板破坏增多 Oligoclonal expansions of GP-specific CD4+T cells Fogarty PF, et al. Clin Adv Hematol Oncol 2003;1:365-71 T-cell activation in ITP Semple et al. Blood 1991;78:2619-25 Semple et al. Blood 1996;87:4245-54 Lost of T-cell tolerance to self antigen in ITP Peng，et al. Blood 2003；101：2721-26 Zhang，et al. J Thrombosis Haemostasis 2007；6:158–65 Disturbed apoptosis of T cells Olsson, et al. Thromb Haemost 2005;93:139-44 Loss of T-cell tolerance 发病机制 对自身抗原免疫耐受缺失 血小板生成减少 血小板破坏增多 Platelet production is suboptimal in ITP patients Autologous 111In-platelet studies show platelet production≤ normal in 2/3 patients TPO levels normal in 75% of ITP patients (relative TPO deficiency) Autoantibodies inhibit both Mk growth and Mk apoptosis Trail-mediated megakaryocyte para-apoptosis leading to in vitro dysmegakaryocytopoiesis and impaired platelet production Thrombopoietin levels in ITP patients ????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????? ? ? ?? Hou et al. Br J Haematol 1998;101:420-4 Decreased platelet production Trail-mediated megakaryocyte para-apoptosis leading to in vitro dysmegakaryocytopoiesis plasma antibody Removal of antibody The number of megakaryocytes Megakaryocyte apoptosis Antibodies inhibit the Generation of megakaryocytes Yang, et al. Blood 2010;116:4307-16 发病机制 对自身抗原免疫耐受缺失 血小板生成减少 血小板破坏增多 Increased platelet destruction Autoantibody-mediated platelet clearance Zucker-Franklin, et al. N Engl J Med 1977;297:517-23 CTL-mediated platelet lysis Olsson, et al.