医学
非典型溶血尿毒综合征
微血管病性溶血性贫血
伊库利珠单抗
ADAMTS13号
血栓性微血管病
补体系统
免疫学
自身抗体
弥漫性血管内凝血
替代补体途径
血栓性血小板减少性紫癜
抗体
内科学
疾病
血小板
作者
Min-Hua Tseng,Shih‐Hua Lin,Jeng-Daw Tsai,Mai‐Szu Wu,I-Jung Tsai,Yeu‐Chin Chen,Min-Chih Chang,Wen‐Chien Chou,Yee-Hsuan Chiou,Chiu‐Ching Huang
标识
DOI:10.1016/j.jfma.2022.10.006
摘要
Atypical hemolytic uremic syndrome (aHUS), characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury, is a rare but life-threatening systemic disorder caused by the dysregulation of the complement pathway. Current advances in molecular analysis and pathogenesis have facilitated the establishment of diagnosis and development of effective complement blockade. Based on this recent consensus, we provide suggestions regarding the diagnosis and management of aHUS in Taiwan. The diagnosis of aHUS is made by the presence of TMA with normal ADAMTS13 activity without known secondary causes. Although only 60% of patients with aHUS have mutations in genes involving the compliment and coagulation systems, molecular analysis is suggestive for helping establish diagnosis, clarifying the underlying pathophysiology, guiding the treatment decision-making, predicting the prognosis, and deciding renal transplantation. Complement blockade, anti-C5 monoclonal antibody, is the first-line therapy for patients with aHUS. Plasma therapy should be considered for removing autoantibody in patients with atypical HUS caused by anti-CFH or complement inhibitor is unavailable.
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