Experience with Haemate P in von Willebrand’s Disease in Adults

Abstract

The virally inactivated pasteurized FVIII concentrate Haemate P contains nearly intact vWF multimers. It is currently the treatment of choice to achieve satisfactory hemostasis for moderate to severe vWD and for patients with variants of vWD that cannot be adequately treated with DDAVP or for whom DDAVP is contraindicated. Therefore, we treated patients with type la, type IIa, type IIb and type III vWD with Haemate P. A correction of the hemostatic defect was seen in all patients. The type of bleeding events included 24 gastrointestinal, 18 other mucosal, 5 central nervous system, 15 orthopedic and other. 28 dental surgical procedures, 9 operative deliveries, tonsillectomies, 17 orthopedic and 11 miscellaneous surgeries were performed under the cover of Haemate P.

Clinical efficacy of highly purified, doubly virus‐inactivated factor VIII/von Willebrand factor concentrate (Fanhdi®) in the treatment of von Willebrand disease: a retrospective clinical study

“…Despite the fact that patients with vWD have been treated with plasma-derived FVIII/vWF concentrates for more than 20 years, only reports of small retrospective series are mainly available in the literature [18][19][20][21]. Therefore, a retrospective study such as this on the use of a FVIII/vWF concentrate in the treatment of vWD is still significant.…”

An update on type 2B von Willebrand disease

“…Grade C, level IV [148,156] Treatment of major bleeding and prophylaxis for major surgery • All treatment plans should be based on objective laboratory determination of response of VWF:RCo and FVIII activity levels to DDAVP or to VWF concentrate infusion. Grade B, level IIb [146,147,150,151,118,[157][158][159][160][161][162][163][164][165][166][167] • Whenever possible, all major surgeries and bleeding events should be treated in hospitals with a 24-h/day laboratory capability and with clinical monitoring by a team including a hematologist and a surgeon skilled in the management of bleeding disorders. Grade C, level IV • For severe bleeding (e.g., intracranial, retroperitoneal) or for prophylaxis of major surgery, initial target VWF:RCo and FVIII activity levels should be at least 100 IU/dl.…”

“…Subsequent dosing should maintain VWF:RCo and FVIII levels above a trough of 50 IU/dl for at least 7-10 days. Grade B, level III [146,147,150,151,[162][163][164][165][166][167] • To decrease risk of perioperative thrombosis, VWF:RCo levels should not exceed 200 IU/dl, and FVIII activity should not exceed 250 IU/dl. Grade C, level IV [131,132,168] • For major surgical procedures in selected patients with type 3 VWD or AVWS who are at risk for poor VWF recovery because of inhibitors, a pre-operative trial infusion of VWF concentrate with pharmacokinetic laboratory monitoring should be considered.…”

Continuous Infusion of Von Willebrand Factor and Factor Viii After Elective Heart Surgery in a 12-Year-Old Girl With Von Willebrand Disease Type 3

“…In the past, it has been recommended for major surgery in patients with type 3 VWD to infuse factor concentrates, such as Haemate P, with a bolus dose of 30± 40 IU/kg, followed by 15± 25 IU/kg every 12 h for 3 days and 15± 25 IU/kg daily for 3± 5 days [8]. Haemate P was also recommended at a dose ranging from 20 to 80 IU/kg every 12 h for periods ranging from 3 to 10 days, depending on the VWD type, the severity of the bleeding, or the type of surgery [9,10]. Continuous infusion of factor concentrates is a new and feasible procedure in situations requiring hemostatic control of pediatric and adult patients with VWD.…”