PROPHYLAXIS THERAPY IN HAEMOPHILIA A: CURRENT SITUATION IN SPAIN

Felix Lucia (1) , José Antonio Aznar (2) , Lydia Abad-Franch (2) , Rebeca Rubio Escuin (1) , Victor Jimenez-Yuste (3) , Rosario Perez Garrido (4) , Francisco Javier Batlle (5) , Ina Balda (6) , Gabriela Alperovich (7) , Rafael Parra (8)

1 Servicio de Hematologia
2 Unidad de Trombosis y Hemostasia
3 Haematology
4 Hospital Universitario Virgen del Rocío [Sevilla]
5 Haematology
6 Servicio de Hematologia y Hemoterapia
7 Medical Department
8 Haemophilia Unit

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 (HB)); 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%). Based on the 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 versus 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 suggests 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.

Mots clés: Medicine

Felix Lucia, José Antonio Aznar, Lydia Abad-Franch, Rebeca Rubio Escuin, Victor Jimenez-Yuste, et al.. PROPHYLAXIS THERAPY IN HAEMOPHILIA A: CURRENT SITUATION IN SPAIN. Haemophilia, 2010, 17 (1), pp.75. ⟨10.1111/j.1365-2516.2010.02378.x⟩. ⟨hal-00599532⟩