Coagulopathy in amyloidosis: combined deficiency of factors IX and X

Am J Hematol. 1977;3:225-35. doi: 10.1002/ajh.2830030303.


Combined severe deficiencies of blood clotting factors IX and X were observed in 2 patients who suffered from systemic amyloidosis. This unique deficiency state was marked by refractoriness to Vitamin K as well as to transfusion therapy. Increased antithrombin activity was present in both individuals and corresponded in time to the emergence of a monoclonal IgG kappa light chain paraprotein in 1. Both patients demonstrated profound bleeding disorders. It is hypothesized that the Vitamin K dependent factors have special affinity for amyloid deposits due to an unusual amino acid (gamma-carboxyglutamic acid) present in these factors.

Publication types

  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adult
  • Amyloidosis / complications*
  • Amyloidosis / pathology
  • Blood Coagulation Disorders / complications*
  • Blood Coagulation Tests
  • Factor X Deficiency / complications*
  • Factor X Deficiency / pathology
  • Female
  • Hemophilia B / complications*
  • Hemophilia B / pathology
  • Humans
  • Hypoprothrombinemias / complications*
  • Middle Aged