Von Willebrands Disease (vWD) is the most common inherited bleeding disorder. We studied the efficacy and feasibility of treating a child with type III severe vWD solely with cryoprecipitate prepared by repeated d-arginine vasopressin (DDAVP)-stimulated plasma exchange donation from a single, dedicated, paternal donor. An infant presented with massive hemorrhage at circumcision. The child was found to have FVIII:C 4 percent, FVIII:Ag 20 percent, vWF:RCo 21 percent, vWF:Ag 3 percent, indicative of severe vWD. His father carried an allele with a defect at the level of vWF mRNA expression but had a negative bleeding history with normal coagulation values. Cryoprecipitate was prepared from serial DDAVP-stimulated plasma exchange donation using peripheral venous access, ACD-A anti-coagulant, and autologous cryoprecipitate- depleted plasma as replacement fluid. Exchange volume was 9,620 ? 2,191 (m ? SD, range: 4,715 to 13,500) ml during the course of 37 plasma exchange donations that the donor has made during the 12 years since the childs birth. Repeated plasma exchange donation was well tolerated, with adverse effects includ-ing mild headache and flushing due to the DDAVP, and citrate toxicity. Cryoprecipitate was stored for less than 1 to 102 months at 70C. Ninety-four percent of the cryoprecipitate was transfused after one year of storage, with a mean collection to transfusion interval of 2.7 years. Cryoprecipitate tested after 13 to 77 months of storage showed 48 to 124 percent of the original FVIII activity; decreased activity was noted with increasing length of storage. Over 12 years, 101,288 units of FVIII were collected in this manner, with a mean content of 202 ? 57 units of FVIII per bag. All bleeding episodes were successfully managed with cryoprecipitate derived from this single donor. Manufacture of plasma exchange donation-derived FVIII resulted in an estimated 50 percent cost reduction compared with similar doses of commercial factor concentrates. Cryoprecipitate prepared by repeated plasma exchange donation of a vWD carrier provided excellent hemostatic function, even after prolonged storage intervals of more than 1 year. Plasma exchange donation of a committed donor may be the safest option for long term management of vWD, and provides a cost effective alternative to commercial factor concentrates
