Multimodal management of refractory gastrointestinal bleeding in myeloma-associated acquired von Willebrand syndrome: a role for emicizumab prophylaxis.

We report the first case of emicizumab prophylaxis in refractory acquired von Willebrand syndrome (AVWS) secondary to multiple myeloma (MM). A 72-year-old woman with IgG kappa MM and gastrointestinal angiodysplasia suffered recurrent life-threatening hemorrhage unresponsive to multimodal therapies, including factor concentrates, intravenous immunoglobulin, plasmapheresis, and endoscopic interventions. Subcutaneous emicizumab combined with octreotide, ongoing anti-myeloma therapy, and previous endoscopic control achieved complete cessation of bleeding despite persistently undetectable von Willebrand factor activity. Over 12 months of follow-up, the patient maintained a very good partial response and remained transfusion-free with an ECOG performance status of 0. This case suggests that emicizumab-mediated restoration of secondary hemostasis can provide sustained bleeding control in refractory AVWS when combined with a multidisciplinary approach, offering a valuable therapeutic option when conventional hemostatic measures fail.