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The HemoRec Database as an Example of a Rare Diseases Registry


(Dz. U. 2002 No. 101 item 926, as amended). The distribution of diagnoses is shown in Table 1, in comparison with the central registry. Data on bleeding episodes were transferred from home diaries of the patients. As of 2 November 2010, 4,895 bleeds were registered, occurring in 314 patients with haemophilia.


The current and future status of the HemoRec database in Poland was presented and discussed recently.8


Currently, HemoRec is used


in a very limited range, individually at each HTC and most commonly for research purposes. In the future, the database could be developed into an interactive platform of communication between patients and HTC workers. It can enable the supervision of home treatment, the dosage of factor concentrates and other aspects of therapy. It could theoretically be integrated with the national registry in the future or replace the current central database, offering much smaller technical capacities than HemoRec. Another possible development is the implementation of the database in the planned network of outpatient clinics for thrombosis and bleeding disorders as an electronic system of documentation. However, entering data into HemoRec and exploring further possibilities given by this database requires significant human and financial resources and good technical support and training.


Conclusion and Discussion


The need for well-designed, informative databases of rare disorders is clearly defined. Accurate and easily accessible data serve patients, physicians, researchers and pharmaceutical companies. The centralisation of information on rare disorders should be perceived not only on the national level, but also on the European level. International databases of rare disorders would facilitate and improve public health surveillance.


A good example of an international registry of rare diseases is the online database of primary immunodeficiencies led by the European Society for Immunodeficiencies (www.esid.org/esid_registry.php). Data collected in the registry enable research and contribute to the improvement of diagnostics, treatment and understanding of the mechanisms of these conditions.16,17


HemoRec creates a good basis


for a national and international database of haemophilia and related bleeding disorders. It already contains data on a representative group of patients with haemophilia and data on the vast majority of Polish patients with haemophilia complicated by the presence of factor VIII/factor IX inhibitors.


Recording all bleeding information (transferring it from the home diaries patients) would provide exact, prospective data on the usage of factor concentrates, frequency and localisation of bleeds and duration of treatment. The small number of bleeding episodes registered in HemoRec results from poor access to source data (individual paper diaries of the patients) and the time-consuming process of transferring the data to the database. The MyHemoRec module could facilitate these tasks, although it requires that the patient has a computer and Internet access.


In summary, we want to highlight the importance of regional and international registries, especially in the field of rare diseases. Launching the HemoRec project in Poland and other countries of the EU could be the first step to creating a modern, European database of inherited bleeding disorders, provided that adequate logistical and financial resources are allocated. n


EUROPEAN ONCOLOGY & HAEMATOLOGY


Figure 1: Polish Haemophilia Treatment Centres Participating in the HemoRec Project from 2006 to 2010


3 12 1 13 6 10 11 2 4 14 7 5 15 8 9


1. Department of Paediatrics, Haematology and Paediatric Oncology, Pomeranian Medical Academy, Szczecin; 2. Department of Haematology, Blood Neoplasms and Bone Marrow Transplantation, Medical University, Wrocław; 3. Department of Haematology and Transplantology, Medical University, Gdansk; 4. Department of Bone Marrow Transplantation, Oncology and Paediatric Haematology, Medical University, Wrocław; 5. Department of Haemato-oncology and Bone Marrow Transplantation, Medical University, Lublin; 6. Department of Internal Diseases and Haematology, J Strus Hospital, Poznan; 7. Department of Haematology, Jagiellonian University, Krakow; 8. Institute of Haematology and Blood Transfusion, Warsaw; 9. Department of Paediatrics, Haematology and Oncology, Medical University, Warsaw; 10. Department of Haematology and Internal Diseases, Regional Medical Centre for Mother and Child, Poznan; 11. Department of Haematology, Medical University, Łodz; 12. Voivodeship Specialistic Hospital, Olsztyn; 13. Department of Haematology, Military Hospital, Szczecin; 14. Department of Paediatrics, Haematology and Oncology, Zabrze; 15. Haematology Department, Holycross Cancer Centre, Kielce.


Table 1: Patients Registered in HemoRec and in the Polish National Registry*


Diagnosis Number of Patients Number of Patients


Registered in HemoRec Registered in the (% of all Diagnoses)


Haemophilia A Haemophilia B Von Willebrand disease


Acquired haemophilia


834 (75.8) 134 (12.2) 54 (4.9)


35 (3.2)


Factor VII deficiency 29 Factor XI deficiency Factor V deficiency Factor X deficiency


1 0 1


Factor XIII deficiency 0 Factor XII deficiency 11 Fibrinogen defects Rare clotting factor


1 43 (3.9)


deficiencies – total Platelet bleeding disorders


Vascular bleeding


disorders Other


Total number of diagnoses


Diagnosis unknown Total number of patients


0 0 0 1,100** 61 1,110***


2,213 (49.7) 387 (8.7)


1,187 (26.7)


34 (0.8) 193 33 20 15 10


210 53


534 (12) 63 30 5 4,453 1 4,454


*Status as of 2 November 2010; **A total of 1,100 diagnoses were assigned to 1,049 patients of all 1,110 registered in HemoRec (44 patients had more than one diagnosis); ***In 61 patients the diagnosis was not specified.


149


National Registry (% of all Diagnoses)


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