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Tail Vein Transection Bleeding Model in Fully Anesthetized Hemophilia A Mice

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Haemophilia in Spain.

J A Aznar¹, F Lucía, L Abad-Franch

¹Congenital Coagulopathies Unit, University Hospital La Fe, Valencia, Spain. aznar_jan@gva.es

Haemophilia : the Official Journal of the World Federation of Hemophilia

|May 13, 2009

Summary

This study surveyed 2400 Spanish patients with haemophilia A (HA) and B (HB), finding significant complications like arthropathy and infections. Prophylaxis reduced bleeding episodes, highlighting the need for improved care for haemophilia patients in Spain.

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Area of Science:

* Hematology
* Epidemiology
* Public Health

Background:

* Haemophilia A (HA) and B (HB) are rare inherited bleeding disorders.
* Comprehensive epidemiological data and healthcare network characterization are crucial for optimizing patient care.
* Previous studies on haemophilia prevalence and complications in Spain are limited.

Purpose of the Study:

* To determine the prevalence of HA and HB in Spain.
* To characterize clinical and genetic features, treatment, and complications in haemophiliac patients.
* To evaluate the healthcare network supporting haemophiliac patients in Spain.

Main Methods:

* A cross-sectional, multi-centre study involving 2400 haemophiliac patients (2081 HA, 319 HB) in active follow-up in Spanish hospitals by December 2006.
* Data collection included clinical and genetic characteristics, treatment, and complications.
* Analysis of disease severity, genetic screening, treatment modalities (factor concentrates, prophylaxis), bleeding episodes, arthropathy, infections (HIV, HCV), and inhibitor development.

Main Results:

* HA prevalence was 86.7%, HB 13.3%. Severe illness affected 32.3% of patients.
* Genetic screening was performed in 32.6%. Coagulation factor concentrates were used by 60% in 2006.
* Prophylaxis was received by 45.8% of severe patients; those on prophylaxis had fewer bleeding episodes (1.3 vs. 4).
* Complications included arthropathy (30%), HIV (16.8%), HCV (34.8%), and inhibitors (10% in severe HA, 6.5% in severe HB).
* Immune tolerance induction therapy was initiated in 34 patients.

Conclusions:

* This study provides the first comprehensive epidemiological overview of haemophilia in Spain.
* Findings highlight significant complications and varying treatment uptake, underscoring the need for evidence-based guidance.
* Results enable comparisons with other countries and assessment of care quality, aiming for equitable improvement of haemophilia care in Spain.