Martínez-Murillo C

Language: Spanish
References: 12
Page: 61-62
PDF size: 577.74 Kb.

ABSTRACT

Von Willebrand disease (VWD) is a hereditary bleeding disorder characterized by a quantitative or qualitative defect of von Willebrand factor (VWF). It presents from 0.1 to 1.3% of the general population; however, many subjects are asymptomatic despite having low concentrations of von Willebrand factor. Symptomatic subjects are presented in 1 per 10,000 live births (0.01% of the population). Women from 5 to 20% are diagnosed by history of menorrhagia. The disease is characterized by mucocutaneous hemorrhages of variable intensity and tends to be fluctuating, being more intense in children and adolescents. The determination of the von Willebrand factor: Antigenic (FvW: Ag) and the cofactor of ristocetin (FvW: RiCo) are the basic tests for Von Willebrand disease, additional studies such as platelet aggregation induced by ristocetin (RIPA) and the study of multimers allow to characterize the VWD for an appropriate treatment. The treatment is usually to correct the presence of hemorrhage or prevent it in the case of a surgical procedure. The choice of treatment depends on the subtype of the Von Willebrand disease and the nature of the hemorrhagic diathesis; the ones of choice are desmopressin (DDAVP) and transfusion therapy with blood products. Among the adjuvant treatments are fibrinolysis inhibitors, oral estrogen-progestagens preparations and adhesive fibrins.

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