A Macroglobulin with Inhibitory Activity Against Coagulation Factor VIII

Deficiency of coagulation factor VIII occurred in a patient with a monoclonal gamma-M protein and characteristic features of Waldenström’s macroglobulinemia. The protein, found to contain lambda light chains, had specific activity against normal factor VIII that was reversible by reduction with 2-m...

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Bibliographic Details
Published in:Blood 1970-03, Vol.35 (3), p.370-376
Main Authors: Castaldi, P.A., Penny, R.
Format: Article
Language:English
Subjects:
Adsorption
Blood Coagulation Disorders - blood
Blood Coagulation Disorders - etiology
Blood Coagulation Tests
Blood Transfusion
Chlorambucil - therapeutic use
Clot Retraction
Factor VIII - antagonists & inhibitors
Factor VIII - metabolism
Female
Humans
Immunoglobulin M - metabolism
Macroglobulins - metabolism
Mercaptoethanol - pharmacology
Mercaptoethanol - therapeutic use
Middle Aged
Nitrogen Mustard Compounds - therapeutic use
Penicillamine - therapeutic use
Penicillins - therapeutic use
Plasmapheresis
Platelet Adhesiveness
Waldenstrom Macroglobulinemia - immunology
Waldenstrom Macroglobulinemia - therapy
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Summary:Deficiency of coagulation factor VIII occurred in a patient with a monoclonal gamma-M protein and characteristic features of Waldenström’s macroglobulinemia. The protein, found to contain lambda light chains, had specific activity against normal factor VIII that was reversible by reduction with 2-mercaptoethanol. Spontaneous correction of the clotting factor deficiency occurred during treatment. It is suggested that the macroglobulin in this patient removed or masked factor VIII by adsorption and that alteration of the protein in vitro by mercaptoethanol and in vivo by treatment removed this capacity.
ISSN:0006-4971
1528-0020
DOI:10.1182/blood.V35.3.370.370