Factor V and VIII deficiency treated with therapeutic plasma exchange prior to redo mitral valve replacement.
A 33-year-old male was admitted to the hospital for a repeat mitral valve replacement. The original surgery, performed in India in 2008 due to rheumatic heart disease, required massive amounts of plasma replacement during and after the surgery. The patient was admitted to our hospital with extremely low Factor V and Factor VIII activities due to a rare combined Factor V and Factor VIII deficiency. His clinical condition on admission was grave due to severe pulmonary hypertension. It was decided to replace the patient's Factor V using therapeutic plasma exchange (TPE) with fresh frozen plasma (FFP) just prior to surgery, and his Factor VIII with Factor VIII concentrate. The patient tolerated the valve replacement surgery very well, without excessive bleeding, and received several more TPE procedures postoperatively. He was successfully made replete with both coagulation factors with little to no bleeding during the procedure and postoperatively. TPE is a promising modality for the treatment of patients with similar factor deficiencies for which a specific factor concentrate is not available, especially those at risk of fluid overload from plasma transfusion. J. Clin. Apheresis 32:196-199, 2017. © 2016 Wiley Periodicals, Inc.
Department of Pathology and Laboratory Medicine, Kansas University Medical Center, Kansas City, Kansas.
Laura
http://orcid.org/0000-0002-9012-6680
L
Brown
Department of Pathology and Laboratory Medicine, Kansas University Medical Center, Kansas City, Kansas.
Lowell
L
Tilzer
Department of Pathology and Laboratory Medicine, Kansas University Medical Center, Kansas City, Kansas.
Fred
F
Plapp
D000328:Adult; D016063:Blood Loss, Surgical / Q000517:prevention & control; D005166:Factor V Deficiency / Q000628:therapy*; D019918:Heart Valve Prosthesis Implantation; D006467:Hemophilia A / Q000628:therapy*; D006801:Humans; D008297:Male; D010951:Plasma Exchange / Q000379:methods*; D011300:Preoperative Care; D012086:Reoperation; D012214:Rheumatic Heart Disease / Q000628:therapy
combined factor V and VIII deficiency; hemophilia; hemostasis; therapeutic plasma exchange