Successful treatment with recombinant factor VIIa of therapy-resistant severe bleeding in a patient with acquired von Willebrand disease.
We describe an elderly man who presented with life-threatening hematuria and gastrointestinal bleeding caused by acquired von Willebrand disease associated with monoclonal gammopathy of undetermined significance (MGUS). Standard therapy with desmopressin, von Willebrand factor-containing factor VIII concentrate, tranexamic acid, and immunoglobulin failed to achieve adequate hemostasis. However, treatment with recombinant activated factor VII (rFVIIa) arrested the bleeding completely. Since acquired von Willebrand disease can lead to life-threatening hemorrhage, clinicians should consider rFVIIa as an effective treatment option if standard therapy fails.
Friederich
P W
PW
Department of Vascular Medicine, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands. p.w.friedrich@amc.uva.nl
Wever
P C
PC
Briët
E
E
Doorenbos
C J
CJ
Levi
M
M
D000368:Aged; D000918:Antibody Specificity; D001327:Autoimmune Diseases / Q000150:complications* / Q000276:immunology; D015942:Factor VIIa / Q000235:genetics / Q000627:therapeutic use*; D006470:Hemorrhage / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D007145:Immunoglobulin kappa-Chains / Q000276:immunology; D008297:Male; D010265:Paraproteinemias / Q000150:complications* / Q000276:immunology; D010266:Paraproteins / Q000276:immunology; D010314:Partial Thromboplastin Time; D011994:Recombinant Proteins / Q000627:therapeutic use; D014842:von Willebrand Diseases / Q000150:complications* / Q000276:immunology; D014841:von Willebrand Factor / Q000276:immunology*
D007074:Immunoglobulin G; D007145:Immunoglobulin kappa-Chains; D010266:Paraproteins; D011994:Recombinant Proteins; D014841:von Willebrand Factor; D015942:Factor VIIa