成人原发免疫性血小板减少症诊治中国专家共识修订版解读.pptxVIP

提要概述诊疗要点疾病分期治疗原则疗效判断 概述取得性本身免疫性出血性疾病占出血性疾病1/3，老年人高发以皮肤黏膜出血为主患者可有明显乏力症状 Average annual ITP incidence by age group and gender (n?=?1145) Life Quality in Chronic ITP PatientsHealthyGeneral PopulationCancerHypertensionArthritisITPITPDiabetesLimoCHFMcMillan et al. American Journal of Hematology DOI 10.1002/ajh/20992 临床体现出血症状一般与血小板计数负有关部分重度血小板降低患者无出血症状或仅轻度出血老年患者出血发生率明显高于年轻患者注意：乏力与血栓形成 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岁头面部其他部位鼻衄/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、连续不止不伴贫血伴有贫血危及生命?1√?√??????2?√?√?????3????√√???4??????√??5???????√√王琳 侯明.原发免疫性血小板降低症出血评分系统临床应用分析。 中华血液学杂志。2023 发病机制对本身抗原免疫耐受缺失血小板生成降低血小板破坏增多 Oligoclonal expansions of GP-specific CD4+T cells Fogarty PF, et al. Clin Adv Hematol Oncol 2023;1:365-71T-cell activation in ITPSemple et al. Blood 1991;78:2619-25Semple et al. Blood 1996;87:4245-54Lost of T-cell tolerance to self antigen in ITPPeng，et al. Blood 2023；101：2721-26 Zhang，et al. J Thrombosis Haemostasis 2023；6:158–65Disturbed apoptosis of T cellsOlsson, et al. Thromb Haemost 2023;93:139-44Loss of T-cell tolerance 发病机制对本身抗原免疫耐受缺失血小板生成降低血小板破坏增多 Platelet production is suboptimal in ITP patientsAutologous 111In-platelet studies show platelet production≤ normal in 2/3 patientsTPO levels normal in 75% of ITP patients (relative TPO deficiency) Autoantibodies inhibit both Mk growth and Mk apoptosisTrail-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 productionTrail-mediated megakaryocyte para-apoptosis leading to in vitro dysmegakaryocytopoiesis plasmaantibodyRemoval of antibodyThe number of megakaryocytesMegakaryocyte apoptosisAntibodies inhibit the Generati