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Diagnosis of Willebrand's disease

Medical expert of the article

Hematologist, oncohematologist
, medical expert
Last reviewed: 06.07.2025

Diagnostic criteria for von Willebrand disease:

  • typical hemorrhagic syndrome;
  • decrease in the specific activity of von Willebrand factor (decrease in vWF:RCo, vWF:CBA, vWF:FVIIIB);
  • for type 2B - positive RIPA with low ristocetin concentrations.

The activity of the von Willebrand factor is related to the ABO blood group. In individuals with blood group 1(0), the content of the von Willebrand factor is constitutionally reduced.

VWF:Ag norms recommended by the World Association of Thrombosis and Hemostasis depending on the blood group

Blood group

Normal vWF:Ag levels

0

36-157%

A

49-234%

IN

57-241%

AB

64-238%

Laboratory diagnostics of von Willebrand disease .

  • Blood test: number of erythrocytes, reticulocytes and hemoglobin; color index, leukocyte formula, ESR; diameter of erythrocytes (on a stained smear);
  • Coagulogram: platelet count and reduction of their adhesion and aggregation; bleeding time and blood clotting time; activated partial thromboplastin and prothrombin time; content of factors IX and VIII and their components (determined by enzyme immunoassay) or disruption of its multimeric structure;
  • Blood biochemistry: total protein, urea, creatinine; direct and indirect bilirubin, transaminases ALT and AST; electrolytes (K, Na, Ca, P);
  • General urine analysis (to exclude hematuria);
  • Fecal occult blood test (Gregersen test);
  • Blood type and Rh factor.

Ultrasound of the abdominal cavity (to exclude hemorrhage in the liver and spleen).

Consultations with a geneticist, hematologist, pediatric gynecologist, ENT specialist, and dentist are required.

Features of laboratory parameters in von Willebrand disease: subnormal thrombocytopenia in peripheral blood; prolongation of bleeding time and normal blood clotting time; violation of the adhesive and aggregation function of platelets.

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