Acquired von Willebrand syndrome can develop as a complication of myeloproliferative neoplasms — including essential thrombocythemia (ET) and polycythemia vera (PV). Managing acute bleeding in this population requires an approach that addresses both the vWF deficiency and the underlying myeloproliferative process.
This protocol is specifically indicated for patients with acquired von Willebrand syndrome arising in the setting of a myeloproliferative neoplasm. In essential thrombocythemia and polycythemia vera, a high platelet mass drives adsorption of HMW multimers — the mechanism behind the acquired vWF defect seen in this population.
Management of acute bleeding episodes in this setting involves hemostatic replacement targeting the vWF deficit, combined with therapy directed at reducing the elevated platelet burden. The complete agent selection, sequencing, and clinical decision algorithm are detailed in the full protocol.
Full regimen, decision criteria, and clinical decision points available below.