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Disorders of Hemostasis

Disorders of Hemostasis

Hemostasis, the process that stops bleeding after a blood vessel injury, is crucial for maintaining the integrity of the circulatory system. However, disorders of hemostasis can disrupt this delicate balance, leading to either excessive clotting or bleeding. These disorders can be broadly classified into thromboembolic disorders and bleeding disorders.
Thromboembolic Disorders
Two factors primarily cause thromboembolic conditions.

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The Swiss Haemophilia Registry-Report From the First 8 Years.

Alessandra Bosch¹, Lorenzo Alberio², Pierre Fontana³

¹Department of Haematology, University Children's Hospital Zurich, Zurich, Switzerland.

Haemophilia : the Official Journal of the World Federation of Hemophilia

|April 21, 2026

Summary

The Swiss Haemophilia Registry (SHR) tracks rare bleeding disorders, showing improved patient outcomes and treatment shifts. Future plans include broader data collection for a comprehensive national resource.

Keywords:

hemophilia A hemophilia B rare diseases registries von Willebrand diseases

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Area of Science:

Hematology
Rare Diseases
Public Health

Background:

Patient registries are crucial for real-world data on rare diseases.
The Swiss Haemophilia Registry (SHR) was established in 2015.
SHR collects data on haemophilia A, B, von Willebrand disease (VWD), and other rare bleeding disorders.

Purpose of the Study:

To describe the bleeding disorder landscape in Switzerland.
To provide insights into the management and treatment of rare bleeding disorders.

Main Methods:

Observational, prospective, longitudinal, multi-centre national registry.
Annual collection of patient demographics, comorbidities, bleeding events, and treatment data.
Data from 929 patients by 2023.

Main Results:

60% haemophilia A, 17% haemophilia B, 15% VWD.
Predominantly male (87%) and adult (75%) cohort with a median follow-up of 5.8 years.
Transition from plasma-derived to extended half-life and non-factor products observed.

Conclusions:

SHR provides real-world evidence of improved haemophilia care and patient outcomes in Switzerland.
The registry documents significant treatment advancements over the past decade.
Future expansion will enhance SHR's value by including more VWD, rare bleeding disorders, and women patients.