Diagnosis and treatment of thrombotic microangiopathy

被引:29
作者
Thonnpson, Gemma L. [1 ,2 ]
Kavanagh, David [1 ,2 ]
机构
[1] Newcastle Univ, Translat & Clin Res Inst, Complement Therapeut Res Grp, Newcastle Upon Tyne, Tyne & Wear, England
[2] Royal Victoria Infirm, Natl Renal Complement Therapeut Ctr, Newcastle Upon Tyne, Tyne & Wear, England
关键词
haemolytic uraemic syndrome; STEC-HUS; thrombotic microangiopathy; thrombotic thrombocytopenic purpura; HEMOLYTIC-UREMIC SYNDROME; THROMBOCYTOPENIC PURPURA; FACTOR-H; C5; INHIBITOR; COMPLEMENT; MUTATIONS; ADAMTS13; DISEASE; GENE; RAVULIZUMAB;
D O I
10.1111/ijlh.13954
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Thrombotic microangiopathy (TMA) is characterized by thrombocytopenia, microangiopathic haemolytic anaemia and end organ damage. TMAs have varying underlying pathophysiology and can therefore present with an array of clinical presentations. Renal involvement is common as the kidney is particularly susceptible to the endothelial damage and microvascular occlusion. TMAs require rapid assessment, diagnosis, and commencement of appropriate treatment due to the high morbidity and mortality associated with them. Ground-breaking research into the pathogenesis of TMAs over the past 20 years has driven the successful development of targeted therapeutics revolutionizing patient outcomes. This review outlines the clinical presentations, pathogenesis, diagnostic tests and treatments for TMAs.
引用
收藏
页码:101 / 113
页数:13
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