von Willebrand disease & TTP

von Willbrand Factor (vWF)

With a prevalence of 0.03 to 0.1% the von Willebrand disease is the most frequent bleeding disorder. The von Willebrand factor (VWF) has two important functions in hemostasis: First, it promotes the adhesion of blood platelets to the injured vessel wall and secondly it functions as a carrier protein for factor VIII and protects it as a chaperone from premature inactivation. The VWF is a glycosylated protein, that is organized in multimers. The size of the VWF multimers is regulated in vivo by a specific plasmatic protease. The lack or a severe reduction of the VWF cleaving metalloprotease ADAMTS-13 is associated with the thrombotic thrombocytopenic purpura (TTP).  

There are three main types of VWD. VWD types 1 and 2 are autosomal dominant traits, while type 3 is inherited autosomal recessive. VWD type 1 is characterized by a quantitative deficiency of the VWF. The VWF is dysfunctional in type 2, and absent in type 3 VWD.

The differential diagnosis of VWD types and subtypes (VWD type 2) requires multiple test, covering most of VWF functions.

 

Typ 1

Typ 2A

Typ 2B

Typ 2M

Typ 2N

Typ 3

VWF:CB

⇩⇩

⇔ / ⇩

⇔ / ⇩

0

VWF:Ag

⇔ / ⇩

⇔ / ⇩

⇔ / ⇩

0

VWF:RCo

⇩⇩

⇩⇩

⇔ / ⇩

0

RIPA

⇔ / ⇩

0

FVIII:C

⇔ / ⇩

⇔ / ⇩

⇔ / ⇩

⇔ / ⇩

⇩⇩

⇩⇩⇩

Multimer

Loss of large multimers

Loss of large multimers

⇔ / supranormal multimers

0

Normal ⇔

Increased ⇧

Reduced ⇩…slightly, ⇩⇩…moderately, ⇩⇩⇩…sever, 0…absent

VWF:CB … Collagen Binding; VWF:Ag ... Antigen; VWF:RCo ... Ristocetin Cofactor, RIPA ... ristocetin-induced platelet agglutination; FVIII:C … FVIII Activity


LITERATURE

TECHNOZYM® vWF:Ag ELISA Kit

vWF:Ag ELISA, calibrator set and controls included.

REF Package Size Insert Lot
5450201 96 tests Download Insert RA73B00, RA81B00

TECHNOZYM® vWF:CBA ELISA Kit

vWF:CBA ELISA, calibrator set and controls included.

REF Package Size Insert Lot
5450301 96 tests Download Insert RB84B00, RB87B00

TECHNOZYM® vWF:CBA ELISA Kit Collagen Type I

vWF:CBA collagen type I ELISA, calibrator set and controls included.

REF Package Size Insert Lot
5450311 96 tests Download Insert RK73B00, RK81B00

TECHNOZYM® vWF:CBA ELISA Kit Collagen Type VI [RUO]

vWF:CBA collagen type VI ELISA, calibrator set and controls included.

REF Package Size Insert Lot
5450321 96 tests Download Insert R181B00, R163B00