Abstract Number: PB1522
Meeting: ISTH 2020 Congress
Background: Von Willebrand disease (VWD) is the most common inherited bleeding disorder, in Algeria the reduced number of patients is an evidence of a screening problem due to the lack of national recommendations and diagnosis guidelines. A large number of testing is necessary to diagnose and classified of VWD.
Aims: Of this study is to diagnose the VWD in Algeria.
Methods: For each patient was performed an activated partial thromboplastin time APTT, Prothrombin time, assay of Factor VIIIc (FVIIIC), assays of von Willebrand factor antigen VWF:Ag (STAGO), ristocetine cofactor VWF:RCo by visual method (SIEMENS), collagene binding test VWF:CB (STAGO) and FVIII binding test VWF:VIIIB (STAGO), blood typing ABO and blood count. The Bleeding severity was determined using ISTH-BAT.
Results: We diagnose 96 patients with VWD. 11% of the patients had VWD type 3, 33% had VWD type 1, 33% had VWD type 2 and 22% had low VWF. The frequency of bleeding family history was 59%, consanguinity was 44%. The ecchymose and epistaxis was the most common bleeding and 97% of patients had bleeding score (BS) ≥ 3. 47% of patients had blood group O. 67% of patients had low level of VWF:RCO (< 30%), 38% had low level of VWF:Ag < 30%) and 29% of patients had low level of VWF:CB (<30%). Distinction between blood groupe O and non O is important in low VWD (VWF ]30-50%]).
Conclusions: The diagnosis of VWD is problematic, being subject to overdiagnosis and underdiagnosis or misdiagnosis. The existence of an isolated prolongation of the TCA or of thrombocytopenia and personal or family bleeding history (ISTH-BAT score ≥ 3) justified the performance of specific tests (FVIIIC, VWF: RCo , VWF: Ag, VWF: CB). The phenotypic diagnosis of Von Willebrand disease is very important for optimal patient management.
To cite this abstract in AMA style:Benlaldj D, Mouedene MA. Diagnosis of von Willebrand Disease in Algeria Laboratory and Clinical phenotype: Single Center Experience [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/diagnosis-of-von-willebrand-disease-in-algeria-laboratory-and-clinical-phenotype-single-center-experience/. Accessed January 21, 2022.
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