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Am J Hematol. 1977;3:225-35.

Coagulopathy in amyloidosis: combined deficiency of factors IX and X.

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 (gamma-carboxyglutamic acid) present in these factors.

PMID:
602938
DOI:
10.1002/ajh.2830030303
[Indexed for MEDLINE]

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