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Coagulopathy in amyloidosis: combined deficiency of factors IX and X.
Am J Hematol 1977; 3:225-35AJ

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.

Authors

No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

602938

Citation

McPherson, R A., et al. "Coagulopathy in Amyloidosis: Combined Deficiency of Factors IX and X." American Journal of Hematology, vol. 3, 1977, pp. 225-35.
McPherson RA, Onstad JW, Ugoretz RJ, et al. Coagulopathy in amyloidosis: combined deficiency of factors IX and X. Am J Hematol. 1977;3:225-35.
McPherson, R. A., Onstad, J. W., Ugoretz, R. J., & Wolf, P. L. (1977). Coagulopathy in amyloidosis: combined deficiency of factors IX and X. American Journal of Hematology, 3, pp. 225-35.
McPherson RA, et al. Coagulopathy in Amyloidosis: Combined Deficiency of Factors IX and X. Am J Hematol. 1977;3:225-35. PubMed PMID: 602938.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Coagulopathy in amyloidosis: combined deficiency of factors IX and X. AU - McPherson,R A, AU - Onstad,J W, AU - Ugoretz,R J, AU - Wolf,P L, PY - 1977/1/1/pubmed PY - 1977/1/1/medline PY - 1977/1/1/entrez SP - 225 EP - 35 JF - American journal of hematology JO - Am. J. Hematol. VL - 3 N2 - 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. SN - 0361-8609 UR - https://www.unboundmedicine.com/medline/citation/602938/Coagulopathy_in_amyloidosis:_combined_deficiency_of_factors_IX_and_X_ L2 - https://onlinelibrary.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0361-8609&date=1977&volume=3&issue=&spage=225 DB - PRIME DP - Unbound Medicine ER -