Haemophilia in Spain.
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.
Congenital Coagulopathies Unit, University Hospital La Fe, Valencia, Spain. aznar_jan@gva.es
J A
JA
Aznar
F
F
Lucía
L
L
Abad-Franch
V
V
Jiménez-Yuste
R
R
Pérez
J
J
Batlle
I
I
Balda
R
R
Parra
V R
VR
Cortina
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D019317:Evidence-Based Medicine; D005169:Factor VIII / Q000627:therapeutic use; D005260:Female; D006467:Hemophilia A / Q000150:complications / Q000453:epidemiology* / Q000235:genetics; D002836:Hemophilia B / Q000150:complications / Q000453:epidemiology* / Q000235:genetics; D006801:Humans; D007108:Immune Tolerance / Q000235:genetics; D007223:Infant; D008297:Male; D008875:Middle Aged; D017410:Practice Guidelines as Topic / Q000592:standards*; D015995:Prevalence; D011788:Quality of Life / Q000523:psychology; D013030:Spain / Q000453:epidemiology; D055815:Young Adult
D005169:Factor VIII