Von Willebrand disease in Finland

Area of Science:

• Hematology
• Clinical Diagnostics
• Genetics

Background:

• The Finnish Red Cross Blood Transfusion Service functions as the national reference laboratory for hemostasis.
• It is the only institution in Finland diagnosing inherited coagulation factor deficiencies.

Purpose of the Study:

• To report on the registered cases of von Willebrand disease (vWD) in Finland.
• To analyze the relationship between vWD type, bleeding severity, and patient demographics.

Main Methods:

• Patient data collection and registration at the national reference laboratory.
• Analysis of diagnostic criteria and bleeding symptom severity.
• Demographic analysis of diagnosed patients.

Main Results:

• 1076 patients with von Willebrand disease (vWD) were registered by September 1997.
• Bleeding symptom severity varied significantly based on vWD type.
• A higher proportion of female patients were diagnosed after the prepubertal phase compared to males.
• The prevalence of severe type 3 vWD was determined to be 4 per 1,000,000.

Conclusions:

• The Finnish national reference laboratory plays a crucial role in diagnosing rare bleeding disorders like vWD.
• Disease presentation and diagnosis timing in vWD are influenced by disease type and sex.
• Type 3 vWD is a rare but severe inherited coagulation disorder.