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Haemophilia in Poland.

J Windyga1, S Lopaciuk, E Stefanska

  • 1Department of Hemostasis and Thrombosis, Institute of Hematology and Blood Transfusion, Warsaw, Poland. windyga@warman.com.pl

Haemophilia : the Official Journal of the World Federation of Hemophilia
|January 18, 2006
PubMed
Summary

This study analyzed 2269 Polish patients with hemophilia A and B, finding severe forms prevalent and significant joint damage in adults. Most severe hemophilia patients over 20 experience arthropathy, highlighting treatment gaps.

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Area of Science:

  • Hematology
  • Orthopedics
  • Public Health

Background:

  • Hemophilia A and B are inherited bleeding disorders with significant global and national prevalence.
  • Severe hemophilia often leads to chronic joint damage and disability, impacting quality of life.
  • Understanding the epidemiological and clinical status of hemophilia patients is crucial for optimizing care.

Purpose of the Study:

  • To analyze the epidemiology of hemophilia A and B in Poland using national registry data.
  • To assess the orthopedic status of severe hemophilia patients and its relation to replacement therapy.
  • To identify current treatment practices and their impact on patient outcomes.

Main Methods:

  • Analysis of data from the Polish National Register of Inherited Bleeding Disorders for 2269 patients.

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  • Clinical and radiological evaluation (Gilbert and Pettersson scales) of 92 severe hemophilia patients.
  • Review of patient history regarding bleeding diathesis, family history, orthopedic surgery, and employment status.

    • Main Results:

    • Prevalence of hemophilia in Poland is ~1:12,300, with severe forms dominating (59.7% A, 56.6% B).
    • Knee joints are most affected in severe hemophilia patients, with 91.3% reporting pain and significant arthropathy.
    • On-demand treatment was common, with no primary prophylaxis; 38% of patients were unemployed.

    Conclusions:

    • Severe hemophilia patients in Poland over 20 years old are universally affected by hemophilic arthropathy.
    • Current treatment approaches, primarily on-demand, are insufficient to prevent severe joint disease.
    • There is a critical need for improved prophylaxis and comprehensive orthopedic management in Polish hemophilia patients.