Multimodal management of refractory gastrointestinal bleeding in myeloma-associated acquired von Willebrand syndrome: a role for emicizumab prophylaxis.
Milan Vujčić, Sanja Madunić, Jasminka Jakelić Piteša et al.
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Case in brief
A 72-year-old woman with IgG κ multiple myeloma, acquired von Willebrand syndrome, and gastrointestinal angiodysplasia had recurrent life-threatening bleeding despite factor concentrates, intravenous immunoglobulin, plasmapheresis, and endoscopic interventions. After subcutaneous emicizumab was added to octreotide, ongoing anti-myeloma therapy, and prior endoscopic control, bleeding ceased despite persistently undetectable von Willebrand factor activity. During 12 months of follow-up, she remained transfusion-free, maintained a very good partial response, and had an Eastern Cooperative Oncology Group performance status of 0.
What made this case unusual
Bleeding control was sustained despite persistently undetectable von Willebrand factor activity, with emicizumab used as prophylaxis in refractory myeloma-associated acquired von Willebrand syndrome.
Diagnostic / clinical pearl
In this case, emicizumab combined with other ongoing measures controlled refractory gastrointestinal bleeding despite undetectable von Willebrand factor activity; clinical bleeding control did not track with recovery of that laboratory measure.
Why it matters
The report describes emicizumab as a potential option when conventional hemostatic measures fail, within a multidisciplinary approach.