title: Treatment of congenital afibrinogenemia with cryoprecipitate collected through a plasmapheresis program using dedicated donors.
abstract: A child with afibrinogenemia was evaluated for prophylactic cryoprecipitate transfusion due to recurrent episodes of traumatic bleeding. The parents would only consider ongoing transfusion therapy if a limited donor program could be established. Automated plasmapheresis was performed on a regular basis on the patient's parents and a limited number of selected donors. Studies on the initial units collected demonstrated that a single 500 mL plasmapheresis yielded a mean of 606 mg fibrinogen in the cryoprecipitate, which was stored in two bags. A total of 166 U of cryoprecipitate from 84 individual plasmapheresis donations were transfused prophylactically every 2-3 weeks over a 16-month period. Compared to transfusions from random donors, the use of a limited number of apheresis donors resulted in a donor exposure reduction of 87%. Clinically, the patient has had minimal bleeding during this period.
authors.affiliation: Department of Pathology, Bonfils Blood Center, Denver, Colorado 80262, USA. hannis.thompson@uchsc.edu
authors.forename: H W
authors.initials: HW
authors.lastname: Thompson
authors.forename: S
authors.initials: S
authors.lastname: Touris
authors.forename: S
authors.initials: S
authors.lastname: Giambartolomei
authors.forename: R
authors.initials: R
authors.lastname: Nuss
mesh_terms: D000347:Afibrinogenemia / Q000151:congenital / Q000628:therapy*; D001782:Blood Donors; D001793:Blood Preservation / Q000379:methods*; D001800:Blood Specimen Collection / Q000379:methods*; D011232:Chemical Precipitation; D015925:Cryopreservation; D006801:Humans; D007223:Infant; D008297:Male; D010956:Plasmapheresis