摘要
免疫性血小板减少症(ITP)为免疫介导的疾病,分为原发性ITP和继发性ITP.结缔组织病(CTD)是一组主要侵犯全身结缔组织的系统性自身免疫性疾病,容易合并继发性ITP的疾病包括系统性红斑狼疮、原发性干燥综合征、抗心磷脂抗体综合征等.CTD-ITP患者出血倾向增加,严重者可致内脏出血,病死率高,是影响CTD患者预后的重要因素.近年来有关CTD-ITP的发病机制、临床表现、治疗方法的研究取得了较大进展,新型生物制剂和促血小板生成药物的使用让更多患者获益.早期诊断、出血程度分级、风险判断以及个性化达标治疗是获得良好预后的关键.
Abstract
Immunological thrombocytopenia(ITP)is an immune-mediated disease,divided into primary ITP and secondary ITP.Connective tissue disease(CTD)is a group of systemic autoimmune diseases that mainly invade the whole body's connective tissue.It is the most common disease to combine with secondary ITP,including systemic lupus erythemato-sus,primary Sjogren syndrome,and antiphospholipid antibody syndrome.CTD-ITP patients have an increased tendency for bleeding,and in severe cases,it can lead to visceral bleeding with a high mortality rate,which is an important factor affect-ing the prognosis of CTD patients.In recent years,significant progress has been made in the research on the pathogenesis,clinical manifestations,and treatments of CTD-ITP.The use of new biological agents and platelet promoting drugs has benefi-ted patients more.Early diagnosis,grading of bleeding severity and risk assessment,as well as personalized and standardized treatment are key to achieve a good prognosis.
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