Coagulopathy in amyloidosis: Combined deficiency of factors IX and X

1 January 1977

journal article
research article
Published by Wiley in American Journal of Hematology

Vol. 3 (3) , 225-235
https://doi.org/10.1002/ajh.2830030303

Abstract

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 (γ-carboxyglutamic acid) present in these factors.

Keywords

This publication has 20 references indexed in Scilit:

Factor IX Deficiency in Gaucher Disease
Archives of internal medicine (1960), 1976
A Comparison of Bovine Prothrombin, Factor IX (Christmas Factor), and Factor X (Stuart Factor)
Proceedings of the National Academy of Sciences, 1974
Amyloidosis with factor X deficiency
The Lancet Healthy Longevity, 1972
The Clinical Challenge of Disseminated Intravascular Coagulation
New England Journal of Medicine, 1970
Acquired Factor IX Deficiency in the Nephrotic Syndrome
Annals of Internal Medicine, 1970
The Genetic Heterogeneity of Hemophilia B
New England Journal of Medicine, 1970
Hageman Factor (Factor XII) in an Affected Kindred and in Normal Adults
British Journal of Haematology, 1968
FACTOR-IX DEFICIENCY IN THE NEPHROTIC SYNDROME
The Lancet, 1967
Amyloidosis Associated with Factor X (Stuart) Deficiency
Annals of Internal Medicine, 1964
Observations on the interaction of phospholipids and certain clotting factors in prothrombin activator formation
Biochimica et Biophysica Acta (BBA) - General Subjects, 1964