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Normalization of plasma factor X levels in amyloidosis after plasma exchange
Author(s) -
Beardell Frank V.,
Varma Mala,
Martinez Jose
Publication year - 1997
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/(sici)1096-8652(199701)54:1<68::aid-ajh10>3.0.co;2-6
Subject(s) - amyloidosis , factor x , medicine , al amyloidosis , fibrinogen , coagulopathy , fibrin , coagulation , amyloid (mycology) , immunoglobulin light chain , pathology , gastroenterology , immunology , thrombin , platelet , antibody
Some patients with systemic light chain amyloidosis develop bleeding complications that can be caused by vascular infiltration with amyloid or by alterations of the coagulation or fibrinolytic systems. Factor X deficiency is the most common cause of bleeding manifestations, although deficiencies of other clotting factors, a disruption in the conversion of fibrinogen to fibrin, and circulating heparin‐like anticoagulants have also been reported. Deficiency of factor X is a well‐recognized cause of bleeding manifestations in patients with light chain amyloidosis. This acquired disorder appears to be secondary to adsorption of factor X to the amyloid fibrils. Previous studies have shown that infusion of plasma into patients with acquired factor X deficiency and amyloidosis induces a transitory improvement of the coagulation tests. However, there is a rapid return to pretransfusion levels. In this manuscript we report the clinical application of plasma exchange in the management of a patient with systemic light chain amyloidosis with acquired factor X deficiency. Am. J. Hematol. 54:68–71, 1997 © 1997 Wiley‐Liss, Inc.