PB1135 - Hemophilia Registry in a province in nothern Spain

Tuesday, July 12, 2022

6:30 PM – 7:30 PM

Location: Exhibition

Presentation Format: In-Person

Co-Author(s)

PL

Paula Lopez

Hematology
HUCA
oviedo, Asturias, Spain

Presenting Author(s)

Isabel Hernández De Castro, MD

doctor
hematology
oviedo, Asturias, Spain

Co-Author(s)

AP

Alberto Porro

Hematology
HUCA
oviedo, Asturias, Spain
JI

Jose Carlos Iglesias

Hematology
HUCA
oviedo, Asturias, Spain
GF

Gabriel Florez Meana

PhD
HOSPITAL UNIVERSITARIO CENTRAL DE ASTURIAS
Oviedo, Asturias, Spain
CR

Carmen Rodriguez de Miñon

PhD
HOSPITAL UNIVERSITARIO CENTRAL DE ASTURIAS
Oviedo, Asturias, Spain
DM

Daniel Martinez Carballeira

PhD
HOSPITAL UNIVERSITARIO CENTRAL DE ASTURIAS
Oviedo, Asturias, Spain
JC

Jose Ramon Corte Buelga

PhD
HOSPITAL UNIVERSITARIO CENTRAL DE ASTURIAS
Oviedo, Asturias, Spain
AB

Angel Bernardo

Hematology
HUCA
oviedo, Asturias, Spain
DM

David Mateos Jimenez

PhD
HOSPITAL UNIVERSITARIO CENTRAL DE ASTURIAS
Oviedo, Asturias, Spain
IS

Inmaculada Soto

Hematology Deparment
Hospital Universitario Central de Asturias
Oviedo, Asturias, Spain

Background: In 2018, the World Federation of Hemophilia (WFH) established the need to create a World Bleeding Disorders Registry (WBDR), a database which aim was to unify the network of hemophilia treatment centers, in order to collect standardized data on hemophiliac patients and guide medical practice.
In 2020 the WBDR has registered 7,208 patients with hemophilia (84% A; 15% B) in 86 treatment centers distributed in 33 countries, with no representation from Spain. The Spanish Ministry of Health has not yet published any hemophilia registry.

Aims: To present a hemophiliac patients’ registry in the Principality of Asturias, first community in Spain to develop such record.

Methods: The Redcap web tool was introduced to create a shared environment that would allow working both with the information already available and with new variables. Such information was loaded and verified from local files to the web registry.

Results: Asturias’ population, according to the latest census of the Spanish National Institute of Statistics, is 1,011,792 inhabitants. Of the 66 hemophiliac patients, with age ranging from 3 to 86 (median 35), 92.4% are type A and 7% type B. 50% of the cases are mild; 6.1% are moderate and 43.9% are severe. 45.5% of them receive prophylactic treatment while 54.5% receive it on demand. The most used ones are DDAVP, Elocta, Nuwiq and Hemlibra, according to the distribution of graph 1. Regarding the development of inhibitor, it was 5% in hemophilia A and 1.5% in hemophilia B.

Conclusion(s): To create a WBDR is not only a mandate from the WFH, but is also necessary to estimate the social and health impact on this population. It aims to improve hemophiliac patients’ quality of life. This is the first registry of hemophilia in the Spanish state, which is currently being extended to the rest of congenital coagulopathies.