HaemophiliaVolume 18, Issue 1 p. e15-e16 LETTERS TO THE EDITORS Liver transplantation in Spanish haemophiliacs J. A. AZNAR, J. A. AZNAR Haemostasis and Thrombosis Unit, University Hospital La Fe, ValenciaSearch for more papers by this authorA. MARCO, A. MARCO Haemostasis and Thrombosis Unit, University Hospital La Fe, ValenciaSearch for more papers by this authorR. PARRA, R. PARRA Haemophilia Unit, University Hospital Vall d′Hebrón, BarcelonaSearch for more papers by this authorV. JIMÉNEZ-YUSTE, V. JIMÉNEZ-YUSTE Coagulopathies Unit, University Hospital La Paz, MadridSearch for more papers by this authorF. LUCÍA, F. LUCÍA Haematology and Haemotherapy Service, University Hospital Miguel Servet, ZaragozaSearch for more papers by this authorI. BALDA, I. BALDA Haematology and Haemotherapy Service, University Hospital Dr. Negrin, Las Palmas de Gran Canaria;Search for more papers by this authorI. SOTO, I. SOTO Haematology and Haemotherapy Service, Hospital Central de Asturias, Oviedo, SpainSearch for more papers by this author J. A. AZNAR, J. A. AZNAR Haemostasis and Thrombosis Unit, University Hospital La Fe, ValenciaSearch for more papers by this authorA. MARCO, A. MARCO Haemostasis and Thrombosis Unit, University Hospital La Fe, ValenciaSearch for more papers by this authorR. PARRA, R. PARRA Haemophilia Unit, University Hospital Vall d′Hebrón, BarcelonaSearch for more papers by this authorV. JIMÉNEZ-YUSTE, V. JIMÉNEZ-YUSTE Coagulopathies Unit, University Hospital La Paz, MadridSearch for more papers by this authorF. LUCÍA, F. LUCÍA Haematology and Haemotherapy Service, University Hospital Miguel Servet, ZaragozaSearch for more papers by this authorI. BALDA, I. BALDA Haematology and Haemotherapy Service, University Hospital Dr. Negrin, Las Palmas de Gran Canaria;Search for more papers by this authorI. SOTO, I. SOTO Haematology and Haemotherapy Service, Hospital Central de Asturias, Oviedo, SpainSearch for more papers by this author First published: 28 September 2011 https://doi.org/10.1111/j.1365-2516.2011.02658.xCitations: 2 J. A. Aznar, Haemostasis and Thrombosis Unit, University Hospital La Fe, Valencia, Spain. Tel: 961973305; fax: 961973052; e-mail: aznar_jan@gva.es Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume18, Issue1January 2012Pages e15-e16 RelatedInformation

The Spanish Epidemiological Study in Haemophilia carried out in 2006 enrolled 2400 patients [2081-86.7% with haemophilia A (HA) and 319-13.3% with haemophilia B]; 465 of them (19.4%) were on prophylaxis. These rates were higher in patients with severe haemophilia (45.4%) and severe paediatric cases (72.5%). On the basis of information recorded in this study, we analysed the current situation of prophylaxis therapy administered to patients with HA in Spain, as well as their orthopaedic status. Prophylaxis was used in 399 (19.2%) patients with HA; such prophylaxis was primary (PP) in 20.3% and secondary (SP) in 75.9% of cases. Among severe HA patients, 313 (45.9%) were on prophylaxis (22.3% on PP and 74.7% on SP). Taking into account the patients' age, 34.7% of severe HA adults were on prophylaxis (6% PP and 92.1% SP), whereas 71.5% of severe HA paediatric patients (40.5% PP and 55.4% SP) received this kind of treatment. Established haemophilic arthropathy (EHA) was detected in 142 from 313 severe HA patients (45.3%) on prophylaxis, but only in 2.9% of patients under PP vs. 59% of patients receiving SP. There was no EHA in adult severe HA patient on PP, whereas 70.4% on SP had joint damage (P < 0.00001). Among paediatric severe HA patients, EHA was detected in 3.3% under PP and 37.8% under SP (P < 0.00001). In conclusion, our data suggest that an early initiation of prophylaxis avoids EHA in the long-term in patients with severe HA. We should emphasize the early onset of prophylaxis regimens.

HaemophiliaVolume 17, Issue 3 p. 542-543 LETTERS TO THE EDITORS Focusing on haemophilia B: prophylaxis in Spanish patients J. A. AZNAR, J. A. AZNAR Haemostasis and Thrombosis Unit, University Hospital La Fe, ValenciaSearch for more papers by this authorJ. F. LUCIA, J. F. LUCIA Department of Haematology, University Hospital Miguel Servet, ZaragozaSearch for more papers by this authorL. ABAD-FRANCH, L. ABAD-FRANCH Haemostasis and Thrombosis Unit, University Hospital La Fe, ValenciaSearch for more papers by this authorR. RUBIO, R. RUBIO Department of Haematology, University Hospital Miguel Servet, ZaragozaSearch for more papers by this authorV. JIMENEZ-YUSTE, V. JIMENEZ-YUSTE Congenital Coagulopathies Unit, University Hospital La Paz, MadridSearch for more papers by this authorR. PEREZ, R. PEREZ Haemophilia Unit, University Hospital Virgen del Rocio, SevillaSearch for more papers by this authorJ. BATLLE, J. BATLLE Department of Haematology, University Hospital Juan Canalejo, A CoruñaSearch for more papers by this authorI. BALDA, I. BALDA Department of Haematology, University Hospital Doctor Negrin, Las Palmas de Gran CanariaSearch for more papers by this authorJ. MUÑOZ-ROBLES, J. MUÑOZ-ROBLES Medical Department, Pfizer SpainSearch for more papers by this authorR. PARRA, R. PARRA Haemophilia Unit, University Hospital Vall d′Hebron, Barcelona, SpainSearch for more papers by this authorON BEHALF OF THE SPANISH HAEMOPHILIA EPIDEMIOLOGICAL STUDY WORKING GROUP, ON BEHALF OF THE SPANISH HAEMOPHILIA EPIDEMIOLOGICAL STUDY WORKING GROUPSearch for more papers by this author J. A. AZNAR, J. A. AZNAR Haemostasis and Thrombosis Unit, University Hospital La Fe, ValenciaSearch for more papers by this authorJ. F. LUCIA, J. F. LUCIA Department of Haematology, University Hospital Miguel Servet, ZaragozaSearch for more papers by this authorL. ABAD-FRANCH, L. ABAD-FRANCH Haemostasis and Thrombosis Unit, University Hospital La Fe, ValenciaSearch for more papers by this authorR. RUBIO, R. RUBIO Department of Haematology, University Hospital Miguel Servet, ZaragozaSearch for more papers by this authorV. JIMENEZ-YUSTE, V. JIMENEZ-YUSTE Congenital Coagulopathies Unit, University Hospital La Paz, MadridSearch for more papers by this authorR. PEREZ, R. PEREZ Haemophilia Unit, University Hospital Virgen del Rocio, SevillaSearch for more papers by this authorJ. BATLLE, J. BATLLE Department of Haematology, University Hospital Juan Canalejo, A CoruñaSearch for more papers by this authorI. BALDA, I. BALDA Department of Haematology, University Hospital Doctor Negrin, Las Palmas de Gran CanariaSearch for more papers by this authorJ. MUÑOZ-ROBLES, J. MUÑOZ-ROBLES Medical Department, Pfizer SpainSearch for more papers by this authorR. PARRA, R. PARRA Haemophilia Unit, University Hospital Vall d′Hebron, Barcelona, SpainSearch for more papers by this authorON BEHALF OF THE SPANISH HAEMOPHILIA EPIDEMIOLOGICAL STUDY WORKING GROUP, ON BEHALF OF THE SPANISH HAEMOPHILIA EPIDEMIOLOGICAL STUDY WORKING GROUPSearch for more papers by this author First published: 11 November 2010 https://doi.org/10.1111/j.1365-2516.2010.02412.xCitations: 6 Dr Jose A. Aznar, Haemostasis and Thrombosis Unit, University Hospital La Fe, Avda. Campanar 21, 46009 Valencia, Spain.Tel.: +34 96 197 3052; fax: +34 96 197 3052;e-mail: aznar_jan@gva.es Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume17, Issue3May 2011Pages 542-543 RelatedInformation

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.

A haemophilia-specific health-related quality-of-life questionnaire (named 'Hemofilia-QoL') was developed to assess quality-of-life in adults with haemophilia, and was psychometrically tested. Seventy-three interviews with haemophilia patients and health care professionals were used to generate the items included in the questionnaire, and expert ratings on the items formulated were used to screen them for potential omission. This was followed by psychometric testing in a sample of 35 patients. Preliminary psychometric testing of the revised questionnaire version, which contains 10 domains (physical health, physical role, joint damage, pain, treatment satisfaction, emotional role, mental health, social support), showed acceptable reliability (alpha = 0.94 for the Hemofilia-QoL total score) and validity, and this will be examined in a subsequent study with a larger patient sample.