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Diagnosis of von Willebrand disease
Last reviewed: 23.04.2024
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Diagnostic criteria for von Willebrand disease:
- a typical hemorrhagic syndrome;
- reduction of specific activity of von Willebrand factor (reduction of vWF: RCo, vWF: CBA, vWF: FVIIIB);
- for type 2B - positive RIPA with low concentrations of ristocetin.
The activity of von Willebrand factor is associated with ABO blood grouping. In individuals with blood group 1 (0), the content of von Willebrand factor is constitutionally reduced.
Standards vWF: Ag, recommended by the World Association of Thrombosis and Hemostasis, depending on the blood type
|
Blood type |
Normal content of vWF: Ag |
|
0 |
36-157% |
|
A |
49-234% |
|
AT |
57-241% |
|
AB |
64-238% |
Laboratory diagnosis of von Willebrand disease .
- Blood test: the number of erythrocytes, reticulocytes and hemoglobin; color index, leukocyte formula, ESR; diameter of erythrocytes (on a stained smear);
- Coagulogram: the number of platelets and reducing their adhesion and aggregation; time of bleeding and clotting time; activated partial thromboplastin and prothrombin time; the content of IX and VIII factors and its components (determined by the enzyme immunoassay method) or a violation of its multimeric structure;
- Biochemical blood test: total protein, urea, creatinine; direct and indirect bilirubin, transaminase ALT and ACT; electrolytes (K, Na, Ca, P);
- General analysis of urine (exclusion of hematuria);
- Analysis of feces for occult blood (Gregersen's test);
- Blood group and Rh-factor.
Ultrasound of the abdominal cavity (to exclude hemorrhage to the liver and spleen).
It is necessary to consult a geneticist, a hematologist, a children's gynecologist, an ENT doctor, a dentist.
Features of laboratory indicators for von Willebrand disease: subnormal thrombocytopenia in peripheral blood; prolongation of bleeding time and normal clotting time; violation of adhesive and aggregation function of platelets.
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