2016共识解读PPT课件.ppt

分值 年 龄 皮肤 黏膜 深部器官 ≥65岁 ≥75岁 瘀 点/瘀 斑／皮下血肿 鼻衄/牙龈出血/口腔血疱／结膜出血 内脏出血（肺、胃肠道、泌尿生殖系统） 中枢 系统 头面部 其他 部位 偶发、可自止 多发、 持续不止 伴有 贫血 不伴贫血 伴有 贫血 危及 生命 1 √ √ 2 √ √ √ 3 √ √ 5 √ √ 8 √ √ ITP 出血评分量表 概述——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 T细胞免疫失耐受 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 ITP患者血小板生成减少 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 血小板生成减少 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 血小板破坏增加 Autoantibody-mediated platelet clearance Zucker-Franklin, et al. N Engl J Med 1977;297:517-23 CTL-mediated platelet lysis Olsson, et al. Nat Med 2003;9:1123-27 GPIbα desialyation leading to platelet apoptosis Heyu Ni, et al. JCI. 2013 on published GPIbα desialyation Kupffer cell PLT PLT Increased platelet destruction Chow, et al. Blood 2010;115:1247-53 GPIIIa knockout mice