伊库利珠单抗
非典型溶血尿毒综合征
医学
微血管病性溶血性贫血
内科学
溶血性贫血
血栓性微血管病
治疗性血浆置换
急性肾损伤
不利影响
贫血
补体系统
胃肠病学
疾病
免疫学
抗体
血栓性血小板减少性紫癜
血小板
作者
Jakub Ruszkowski,Zbigniew Heleniak,Alicja Dębska‐Ślizień
出处
期刊:PubMed
日期:2018-09-21
卷期号:45 (267): 119-121
被引量:2
摘要
Atypical hemolytic uremic syndrome (aHUS) is an extremely rare disease characterized by acute kidney injury, thrombocytopenia, and microangiopathic hemolytic anemia. Majority of patients have an underlying complement abnormality what makes aHUS possible to treat using eculizumab, antibody against the complement protein C5. Up to 2018, Polish adult patients were treated using glucocorticosteroids, plasma exchange or plasma infusion. Unfortunately, such protocols/ interventions were associated with poor prognosis; about 67% of adult patients with aHUS had been progressing to end-stage renal disease or had died within three years after diagnosis. Release of eculizumab significantly improve the prognosis of aHUS. We describe new drug program and case of first included adult patient: after ineffective nonspecific treatment of aHUS with plasma infusions (10 units), plasma exchanges (7 sessions) and glucocorticosteroids, we decided to introduce eculizumab. The clinical and laboratory tests showed effectiveness of the therapy after sixth infusion; no adverse effects were observed.
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