Hoepting, Matthias and Budde, Ulrich and Tiede, Andreas and Grube, Matthias and Hahn, Joachim and Herr, Wolfgang and Heimerl, Susanne and Hart, Christina (2022) Distinct Mechanisms of IgM Antibody-Mediated Acquired von Willebrand Syndrome and Successful Treatment with Recombinant von Willebrand Factor in One Patient. ACTA HAEMATOLOGICA, 145 (4). pp. 454-457. ISSN 0001-5792, 1421-9662
Full text not available from this repository. (Request a copy)Abstract
Acquired von Willebrand Syndrome (AVWS) is a rare coagulation disorder which can be associated with IgM paraproteinaemia. Recently, recombinant von Willebrand factor (rVWF) has become available for the treatment of bleedings in patients with inherited von Willebrand disease, but experience in patients with AVWS is limited. We report on 2 patients with AVWS with underlying IgM paraproteinaemia with distinct underlying pathomechanisms. In 1 patient, the paraprotein built unspecific complexes with von Willebrand factor (VWF). In the other patient, we were able to detect an IgM antibody against VWF. Bleeding in this patient was successfully treated with rVWF. To our knowledge, this is the first report about the successful use of rVWF in a patient with AVWS with the detection of a VWF-specific antibody. (c) 2022 The Author(s). Published by S. Karger AG, Basel
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | ; Bleeding disorders; Paraproteinaemia; Acquired von Willebrand syndrome; von Willebrand factor; Monoclonal gammopathy of undetermined significance |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Innere Medizin III (Hämatologie und Internistische Onkologie) Medicine > Lehrstuhl für Klinische Chemie und Laboratoriumsmedizin |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 09 Jan 2024 09:35 |
| Last Modified: | 09 Jan 2024 09:35 |
| URI: | https://pred.uni-regensburg.de/id/eprint/57102 |
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