Successful use of caplacizumab in a case of refractory acquired thrombotic thrombocytopenic purpura following subacute thyroiditis. (February 2021)

• Record Type: Journal Article
• Authors: Loscocco, Giuseppe G.; Malandrino, Danilo; Vannini, Francesco; Vinci, Maria Costanza; Di Marzio, Giulia; Fallai, Linda; Scappini, Barbara
• Is Part Of: Transfusion and apheresis science. Volume 60:Number 1(2021)
• Publication Date: 2021-02
• Subjects: Subacute thyroiditis -- Acquired thrombotic thrombocytopenic purpura -- Caplacizumab; Blood -- Transfusion -- Periodicals; Hemapheresis -- Periodicals; 615.39
• DOI: 10.1016/j.transci.2020.103010
• Languages: English
• ISSNs: 1473-0502

Abstract

Abstract: 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.