Plasma Product Treatment in Various Types of von Willebrand’s Disease

Abstract

Four different virus-inactivated factor VIII concentrates (Haemate P, Behring; Profilate, Alpha, FVIII-VHP-vWF, CRTS), near-pure von Willebrand factor (Facteur Willebrand, CRTS) or one recombinant FVIII preparation (Recombinate, Baxter) were given to one or more patients with different forms of von Willebrand’s disease. Duke bleeding time, VIII:C, vWF:Ag, RCof activity, and the multimeric pattern of plasma vWF were monitored. Both Duke bleeding time and the multimeric pattern were normalized after treatment with Haemate P, FVIII-VHP-vWF, or Facteur Willebrand, and to a lesser extent after Profilate. Except in one case, the reduction in bleeding time lasted longer after Haemate P than after the other concentrates. Recombinate had no effect on primary hemostasis, and the half-life of VIII:C was very short. If prompt hemostasis is required, and when pharmacological correction of the defect is impossible, we recommend a concentrate containing both FVIII and the full complement of vWF multimers, but for prophylactic treatment pure von Willebrand factor may be used.

DDAVP treatment in a child with von Willebrand disease type 2M

“…Similarly the bleeding time, an important determinant of the bleeding tendency in vWD, in type 2M patients might be borderline or prolonged. The common therapy of choice during operative interventions in rarely occurring subtypes of vWD has been proposed to be the transfusion of vWF/ FVIII plasma concentrates [1,3,5,11]. In this report, however, we describe for the ®rst time the feasibility of perioperative DDAVP administration in a child with vWD type 2M.…”

6 Factor VIII Concentrates, Factor VIII/von Willebrand Factor Concentrates, Factor IX Concentrates, Activated Prothrombin Complex Concentrates

“…These concentrates contain factor VIII as well as hemostatically effective vWf, especially its highly polymerized multimers [12,25].…”

“…It is formed in endothelial cells and alpha granules of platelets and fulfills several functions [12,46,52]: -In primary hemostasis, it connects platelets with collagen of the subendothelial layer of the blood vessel [25]. vWf activity can thus be measured as collagen-binding activity.…”

“…The biological half-life of vWf is 6-12 h; infusion, subcutaneous injection, or nasal application of the vasopressin analogue DDAVP (1-desamino-8-D-arginin-vasopressin; desmopressin) releases vWf and factor VIII from the body's reservoirs and causes an approximately three-fold increase over initial plasma levels. Thus DDAVP can be employed to stop bleeding in mild forms of von Willebrand syndrome (type 1) and in mild hemophilia A during minor bleeding episodes or minor surgery [12,38].…”

“…Additional isolation steps include either immunoaffinity chromatography, ion exchange chromatography or precipitation procedures [1,10,26,44]. Precipitation as well as chromatography lead to enrichment with functional vWf [12,43].…”