VON WILLEBRAND DISEASE IN PREGNANCY

Von Willebrand disease is the most common inherited bleeding disorder, found in approximately 1 of the general population ,without ethnic differences(1,2).VWD is the result of a deficiency or defect in Von Willebrand factor ,the large multimeric protein which mediates platelet adhesion and serves as a carrier protein for factor VIII. There are three major types. Type 1 is the result of a partial ,quantitative deficiency of a structurally normal VWF, and accounts for 70-80 of all VWD patients. Type 2 includes several qualitative defects in VWF that affects its multimeric structure or function. Patients with type3 VWD are homozygous or doubly heterozygous for two mutant VWF alleles ,with a resulting complete deficiency of VWF and a secondary severe deficiency of FVIII. Although the autosomal inheritance pattern predicts that both sexes should be equally affected, there is a higher frequency of symptomatic VWD in women because of the haemostatic challenges of menses, pregnancy and delivery(3-5). Pregnancy in these women requires specialised and individualised management provided by a multidisciplinary team of obstetricians, haematologists and anaesthetists.