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- Title
Daratumumab as a novel treatment for refractory acquired von Willebrand syndrome associated with monoclonal gammopathy.
- Authors
Mathew Thomas, Vinay; Gilreath, Jeffrey A.; Newton, Shawn; Smock, Kristi J.; Lim, Ming Y.
- Abstract
During this admission, he underwent further haematological work-up for the first time which revealed a diagnosis of AVWS with a FVIII:C level of 17% (56-191), VWF antigen [VWF:Ag] level of 16% (56-198) and VWF ristocetin cofactor [VWF:RCo] level of <20% (51-215). Upon completion of therapy, there was a slight improvement in the VWF:Ag levels, but no immediate improvement in the VWF:RCo and FVIII:C activity levels. Notably, he was found to be negative for inhibitors to VWF by VWF GPIbM mixing study (Versiti) and negative for anti-VWF antibodies (IgG or IgM) by ELISA (personal communication, Robert R Montgomery). As he remained anaemic due to presumed occult gastrointestinal bleed, he received iron infusion once and IVIG 1 gm/kg daily × 2 days, with relatively brisk normalization of his VWF parameters (VWF:RCo 111%, VWF:Ag 106%) and FVIII:C 122%; VWF multimeric analysis was normal.
- Subjects
VON Willebrand disease; PURE red cell aplasia; DARATUMUMAB; THERAPEUTICS; BLOOD group incompatibility; IMMUNOGLOBULIN M; AUTOIMMUNE hemolytic anemia
- Publication
Haemophilia, 2021, Vol 27, Issue 4, pe563
- ISSN
1351-8216
- Publication type
Article
- DOI
10.1111/hae.14323