A Confounding Case of Inherited Factor V Deficiency Complicated by Inhibitors at First Presentation

Transfusion Medicine and Hemotherapy : Offizielles Organ Der Deutschen Gesellschaft Fur̈ Transfusionsmedizin Und Immunham̈atologie
Hema SubramanianTarun Kumar Dutta

Abstract

Inherited factor V deficiency / Owren's disease has varied clinical manifestations ranging from asymptomatic laboratory abnormalities to massive hemorrhage. The acquired form due to inhibitors following antibiotic therapy, infection, or surgery is less common, and spontaneous development of inhibitors is not known. An 18-year-old boy presented with bleeding axillary and groin ulcers. At the age of 15, due to recurrent epistaxis and gum bleed, he was diagnosed with acquired factor V deficiency with positive inhibitor screen and treated with fresh frozen plasma (FFP) transfusion and temporary azathioprine. Coagulation workup at his current presentation also revealed acquired factor V deficiency with presence of inhibitors. The tests were repeated after 6 weeks of intermittent FFP transfusion, and the differences observed included negative inhibitor screen and complete correction on mixing studies, but factor V level was 2%. Evidence of inhibitors at presentation favored acquired disease. However, younger age of onset, detection of inhibitors 1 year after first episode of self-regressing bleed, lack of identifiable triggers, and persistent bleeding with reduced factor levels after disappearance of inhibitors favored inherited fact...Continue Reading

Citations

Oct 8, 2020·Transfusion and Apheresis Science : Official Journal of the World Apheresis Association : Official Journal of the European Society for Haemapheresis·Tomasz WasilukPiotr Radziwon

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