Presurgical plasma exchange is ineffective in correcting amyloid associated factor X deficiency.
In patients with rare factor deficiencies, for which no factor concentrates are available, plasma exchange (PE) is an option for raising the desired factor level to approximately 80% for surgery. We report a case of acquired factor X (FX) deficiency due to amyloidosis that required urgent surgical repair of an AV fistula aneurysm. This patient had a FX level of 3% at presentation; after 1.5 volume PE with fresh frozen plasma (FFP), his post-exchange FX was only 5%, indicating rapid adsorption of FX to amyloid fibrils. He was managed successfully with FEIBA during surgery.
Barker
Brad
B
Department of Pathology, University of Texas Southwestern Medical School, Dallas, TX 75390-9073, USA.
Altuntas
Fevzi
F
Paranjape
Geeta
G
Sarode
Ravindra
R
D000682:Amyloid / Q000378:metabolism*; D000686:Amyloidosis / Q000628:therapy; D017541:Aneurysm, False / Q000628:therapy; D001779:Blood Coagulation Factors / Q000627:therapeutic use; D005170:Factor X / Q000096:biosynthesis; D005171:Factor X Deficiency / Q000175:diagnosis* / Q000628:therapy*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010314:Partial Thromboplastin Time; D010951:Plasma Exchange / Q000379:methods*; D011517:Prothrombin Time; D013997:Time Factors
D000682:Amyloid; D001779:Blood Coagulation Factors; D005170:Factor X; C065655:anti-inhibitor coagulant complex