Registry of inherited bleeding disorders in Poland--current status and potential role of the HemoRec database.
We present data collected in HemoRec, an Internet-based platform implemented in 2006 in 15 haemophilia treatment centres in Poland and compare them with the national registry of inherited bleeding disorders established since 1991 at the Institute of Haematology and Blood Transfusion in Warsaw. We also analyse the current status of haemophilia treatment in Poland as well as future perspectives. Data on 1102 patients registered in HemoRec were analysed and compared with 4294 patients in the national registry (status as at 17.08.2009). The number of patients with severe haemophilia, mild/moderate haemophilia and von Willebrand in HemoRec is 530, 328 and 54 (respectively), compared with 1199, 1167 and 1128 in the national registry. The mean age of all haemophilic patients registered in HemoRec is 26.2 years, compared with 37.3 years in the general Polish male population in 2008. The number of haemophilic patients with inhibitor registered in HemoRec is 102 compared with 155 in the national registry (resulting in a prevalence of 14.9% of all severe haemophilia A and 1.6% of all severe haemophilia B patients). HemoRec includes data on a representative group of Polish haemophilic patients, mostly with haemophilia and haemophilia with inhibitor. von Willebrand's disease is largely under-registered in Poland. The survival of Polish haemophilic patients is shorter than that in the general population. The number of inhibitor patients in Poland is relatively large and should be decreased by wider availability of immunotolerance induction in 2010.
Zdziarska
J
J
Department of Hematology, Jagiellonian University School of Medicine, Krakow, Poland. joannaz@patio.strefa.pl
Chojnowski
K
K
Klukowska
A
A
Łętowska
M
M
Mital
A
A
Musiał
J
J
Podolak-Dawidziak
M
M
Windyga
J
J
Ovesna
P
P
Brabec
P
P
Zawilska
K
K
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D025861:Blood Coagulation Disorders, Inherited / Q000453:epidemiology*; D002648:Child; D002675:Child, Preschool; D019992:Databases as Topic / Q000706:statistics & numerical data*; D005260:Female; D006470:Hemorrhage / Q000453:epidemiology; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D011044:Poland / Q000453:epidemiology; D012042:Registries; D055815:Young Adult