LABORATORY DIAGNOSIS OF VON WILLEBRAND DISEASE

Abstract

The aim of the study. To determine the frequency of Willebrand disease (VWD) in patients which turned to the SI IBPTM of the NAMS of Ukraine with hemorrhagic manifestations. Research metods. Object and research methods. The object of the study were 298 people with hemorrhagic syndrome aged from 7 months to 66 years old who have turned to the specialists of the institution since 01.01.2021, of which 198 (66,4%) came in for the first time. Coagulation hemostasis was investigated by methods that meet international standards and recommendations. Ristocetin-cofactor activity of factor Willebrand (VWF: RCo), content of the antigen VWF (VWF: AG), and coagulant activity of factor VIII (FVIII: C) were performed for the diagnosis of VWD. Results and Conclusions. VWD were found in 29 patients (9,7%) of the 298 people with hemorrhagic syndrome. VWD was revealed in 7,6% of patients from the 198 patients who were first investigate. The 15 (51,7%) VWD patients was diagnosed for the first time. In 48 (24,2%) was first diagnosed with hereditary or caused by various factors of disaggregational thrombocytopathy. Von Willebrand disease is the most common type of congenital hemostasis disorders (0,6-1,3% of the population), the type of inheritance is autosomal recessive/dominant. An algorithmic approach should be applied to diagnosis. Laboratory diagnosis consists of screening coagulological tests and three levels special tests. To establish the diagnosis, the necessary tests are determined by VWF: RCo, VWF: Ag and FVIII: C.