Haemophilia in Spain

Abstract

To determine the prevalence of haemophilia A and B and their complications in Spain, and to characterize the health care network providing support to haemophiliac patients. The study examines clinical and genetic characteristics, treatment options, and complications observed during the course of the disease. Cross-sectional multi-centre study. The study population were patients with HA and HB in active follow-up at any Spanish hospital by December 2006. We studied 2400 haemophiliacs, 2081 (86.7%) HA and 319 (13.3%) HB patients. Illness was severe in 32.3% of patients, moderate in 16.4%, and mild in 51.3%. Genetic screening was carried out in 32.6% of the patients. Treatment administered in 2006 consisted of coagulation factor concentrates in 60% of patients. Until December 2006, 45.8% of severely ill patients were taking prophylaxis. The mean number of bleeding episodes in 2006 was four for patients not receiving primary prophylaxis and 1.3 for those taking primary prophylaxis. Thirty percent of patients had established haemophiliac arthropathy in at least one joint; 16.8% of patients were HIV-infected and 34.8% HCV-infected. Inhibitors were detected in 10% of severe HA patients and in 6.5% of severe HB patients. Immune tolerance induction therapy was started in 34 patients. This is the first comprehensive study on the epidemiology of haemophilia in Spain. It will enable us to draw comparisons with neighbouring countries, to assess the quality of care provided to haemophiliacs in Spain, and to provide evidence-based guidance for the even provision and improvement of such care.

Diagnosis and Management ofvon Willebrand Disease in Spain

“…1 A recent Spanish epidemiological study has estimated that 2518 individuals have hemophilia A and 387 have hemophilia B. 2,3 The estimated prevalence of hemophilia, therefore, would be $1 in 7857 males. This estimate is lower than that derived from the extrapolation of data from Canada (1 in 5811) or the United Kingdom (1 in 5143) and similar to that reported from European countries like Sweden (1 in 7374) or Poland (1 in 8492).…”

Hemofilia: ejercicio y deporte

“…Actualmente la esperanza de vida del hemofílico se asemeja a la de la población general y la edad media del paciente hemofílico se sitúa alrededor de los 30 años. Un elevado porcentaje de los pacientes hemofílicos graves presentan lesiones del aparato locomotor y, para centrar la importancia de las necesidades de terapia física, concretamente en España, el 12,4% de los pacientes menores de 14 años presenta artropatía 3 ( fig. 1).…”

“…Los sangrados intraarticulares o hemartrosis son la manifestación clínica más frecuente y más conocida de la hemofilia, tanto grave como moderada, representando el 65-80% de todas las hemorragias 9,10 . Las articulaciones afectadas en mayor proporción son las rodillas, los codos y los tobillos, que suponen el 60-80% de todas las hemartrosis 3,11 . Si estas hemorragias no son tratadas o son mal tratadas, se produce a largo plazo un daño articular irreversible que conduce a la artropatía hemofílica en la segunda década de vida 12,13 .…”

A Review on Hemophilia in Children

“…Hemarthrosis as the predominant presentation with most affected joint being knee joints were also found in Korean study 21 . However many other studies conducted in Netherlands, France and Spain showed ankle joint as mostly affected joints 22,23,24 .…”