Presurgical plasma exchange is ineffective in correcting amyloid associated factor X deficiency.

Abstract

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.

• Authors: Brad Barker, Fevzi Altuntas, Geeta Paranjape, Ravindra Sarode
• Journal: Journal of clinical apheresis (J Clin Apher) Vol. 19 Issue 4 Pg. 208-10 ( 2004) ISSN: 0733-2459 [Print] United States
• PMID: 15597349 (Publication Type: Case Reports, Journal Article)

Chemical References

• Amyloid
• Blood Coagulation Factors
• Factor X
• anti-inhibitor coagulant complex

Topics

• Amyloid (metabolism)
• Amyloidosis (therapy)
• Aneurysm, False (therapy)
• Blood Coagulation Factors (therapeutic use)
• Factor X (biosynthesis)
• Factor X Deficiency (diagnosis, therapy)
• Humans
• Male
• Middle Aged
• Partial Thromboplastin Time
• Plasma Exchange (methods)
• Prothrombin Time
• Time Factors