Hematology Unit, Azienda Ospedaliero-Universitaria Careggi, Florence, Italy; Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
Department of Internal Medicine, Azienda Ospedaliero-Universitaria Careggi, Florence, Italy; Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
Transfus Apher Sci. 2021 Feb;60(1):103010. doi: 10.1016/j.transci.2020.103010. Epub 2020 Nov 7.
Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare condition mainly characterized by microangiopathic hemolytic anemia, thrombocytopenia, reported in approximately three cases per one million adults per year. Some reports describing co-occurrence of aTTP and other autoimmune disorders, as Graves' thyroiditis, are reported. To the best of our knowledge this is the first report describing co-occurrence of subacute thyroiditis and aTTP. The patient was refractory to conventional therapy with plasma exchange, steroids and rituximab but was successfully treated with the addition of caplacizumab, an anti-VWF bivalent variable-domain-only immunoglobulin fragment that inhibits interaction between VWF multimers and platelets.
获得性血栓性血小板减少性紫癜（aTTP）是一种罕见疾病，主要表现为微血管性溶血性贫血、血小板减少症，每年每百万成年人中约有 3 例报告。一些报告描述了 aTTP 与其他自身免疫性疾病同时发生的情况，如格雷夫斯甲状腺炎。据我们所知，这是首例描述亚急性甲状腺炎和 aTTP 同时发生的报告。该患者对血浆置换、类固醇和利妥昔单抗的常规治疗无效，但加用 caplacizumab 后成功治疗，caplacizumab 是一种抗 VWF 二价可变结构域免疫球蛋白片段，可抑制 VWF 多聚体与血小板的相互作用。
