Factor-X deficiency in amyloidosis: a critical review

Abstract

Thirty cases of amyloidosis with factor-X deficiency, including six of our own, were reviewed. Modest deficiency of factor X was often associated with severe bleeding. In many of the cases, clinical bleeding could not be accounted for by deficiency of factor X alone. Other hemostatic defects were found in these patients and probably contributed to the bleeding. Modes of treatment, including the empiric use of corticosteroids and splenectomy, were discussed in light of current knowledge of pathogenesis of this unusual blood clotting disorder. This involves the interaction of amyloid fibrils and blood clotting factors.