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Coagulopathy in amyloidosis: Combined deficiency of factors IX and X
Author(s) -
McPherson Richard A.,
Onstad John W.,
Ugoretz Richard J.,
Wolf Paul L.
Publication year - 1977
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830030303
Subject(s) - coagulopathy , amyloidosis , medicine , immunoglobulin light chain , antithrombin , clotting factor , amyloid (mycology) , aa amyloidosis , immunology , gastroenterology , endocrinology , antibody , pathology , heparin , disease , familial mediterranean fever
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.