Contributions
Type: Publication Only
Background
Considering and realising the remarkable heterogeneity of MPN care in Hungary an MPN Working Group has been established in year 2012.
Aims
The Hungarian MPN Working Group created our MPN Registry in 2013, the aims were: (1) to gain epidemiological, diagnostic, therapeutic data, to follow up complications and disease transformations. (2) to investigate the adherence to the WHO/2008 diagnostic criteria and to the Landolfi therapeutic guidelines, to gain insight into vascular and haematological complications. (3)Try to identify crucial issues and possible gaps, and promote internationally accepted, standard care in MPN.
Methods
The basis of the Hungarian MPN registry was the former Regional Centre of the Hungarian Academy of Sciences, Veszprém. The questionnaire had been thoroughly updated regarding the 2008 WHO diagnostic criteria (morphology, mutations, etc.)with focus on complications, risk stratification and treatment. The electronic platform can be continuously updated as needed by our steering committee (new molecular results e.g. calreticulin mutations can be included). All haematologists using the system are entitled to initiate search and association analysis. Our MPN Registry is legally permitted by our authorities (ETT-TUKEB).
Results
During the two active years of the Hungarian MPN Registry 15 of our major or smaller haematological centers provided patient data, alltogether reaching the evaluable patient number of 820. Even if some major centers data are still missing probably our data are representative enough to characterize MPN patient care in our country. The most important data are summarised in Table.I
The complex multiparametric analysis of vascular events (venous, arterial, minor, major) correlation with haematocrit, cell counts (platelet, leukocyte, monocyte), Landolfi score, traditional vascular risk factors, treatment modalities will be presented in details. We detected some shortcomings regarding the diagnostic evaluation of some JAK2V617F negative PV and some myelofibrosis cases, this will be shown in a separate poster (M. Egyed et al.) Treatment modalities have been analysed in depth, both regarding treatment modalities (i.e. phlebotomy, ASA, cytoreductive drugs, interferons, JAK inhibitors,etc.) and in the context of cell counts, vascular complications and risk stratification. Even if the observation was short, we found interesting transformation patterns in PV (408 patients): myelofibrosis 22, MDS-AML in two patients, secondary lymphoproliferative disease in two patients.
Summary
1. We have created a national MPN Registry covering a large part of Hungary. The database is operational, online, userfriendly, easily adjustable to the new professional needs. It is convenient for complex search, correlation and other multiparametrical analysis. The data collected so far are in concert with the international epidemiological data.
2. In addition, it gives a valuable reflection on the adherence to the diagnostic and therapeutic standards, revealing some heterogenities. The Hungarian MPN Working Group decided to help small centers to render better diagnostic possibilities (rare genetic alterations and determination of von Hippel Lindau mutation). The Steering Committee tries to facilitate patient enrollment to new MPN drug multicenter trials, too.
3. The early results of our database clearly show that important correlations could be made or further confirmed regarding vascular events, cell counts, monocyte number, etc. In spite of the short observation period patients with non-myeloid transformation were also found deserving further attention.
Keyword(s): Aspirin, Polycythemia vera, Transformation, WHO classification
Type: Publication Only
Background
Considering and realising the remarkable heterogeneity of MPN care in Hungary an MPN Working Group has been established in year 2012.
Aims
The Hungarian MPN Working Group created our MPN Registry in 2013, the aims were: (1) to gain epidemiological, diagnostic, therapeutic data, to follow up complications and disease transformations. (2) to investigate the adherence to the WHO/2008 diagnostic criteria and to the Landolfi therapeutic guidelines, to gain insight into vascular and haematological complications. (3)Try to identify crucial issues and possible gaps, and promote internationally accepted, standard care in MPN.
Methods
The basis of the Hungarian MPN registry was the former Regional Centre of the Hungarian Academy of Sciences, Veszprém. The questionnaire had been thoroughly updated regarding the 2008 WHO diagnostic criteria (morphology, mutations, etc.)with focus on complications, risk stratification and treatment. The electronic platform can be continuously updated as needed by our steering committee (new molecular results e.g. calreticulin mutations can be included). All haematologists using the system are entitled to initiate search and association analysis. Our MPN Registry is legally permitted by our authorities (ETT-TUKEB).
Results
During the two active years of the Hungarian MPN Registry 15 of our major or smaller haematological centers provided patient data, alltogether reaching the evaluable patient number of 820. Even if some major centers data are still missing probably our data are representative enough to characterize MPN patient care in our country. The most important data are summarised in Table.I
The complex multiparametric analysis of vascular events (venous, arterial, minor, major) correlation with haematocrit, cell counts (platelet, leukocyte, monocyte), Landolfi score, traditional vascular risk factors, treatment modalities will be presented in details. We detected some shortcomings regarding the diagnostic evaluation of some JAK2V617F negative PV and some myelofibrosis cases, this will be shown in a separate poster (M. Egyed et al.) Treatment modalities have been analysed in depth, both regarding treatment modalities (i.e. phlebotomy, ASA, cytoreductive drugs, interferons, JAK inhibitors,etc.) and in the context of cell counts, vascular complications and risk stratification. Even if the observation was short, we found interesting transformation patterns in PV (408 patients): myelofibrosis 22, MDS-AML in two patients, secondary lymphoproliferative disease in two patients.
Summary
1. We have created a national MPN Registry covering a large part of Hungary. The database is operational, online, userfriendly, easily adjustable to the new professional needs. It is convenient for complex search, correlation and other multiparametrical analysis. The data collected so far are in concert with the international epidemiological data.
2. In addition, it gives a valuable reflection on the adherence to the diagnostic and therapeutic standards, revealing some heterogenities. The Hungarian MPN Working Group decided to help small centers to render better diagnostic possibilities (rare genetic alterations and determination of von Hippel Lindau mutation). The Steering Committee tries to facilitate patient enrollment to new MPN drug multicenter trials, too.
3. The early results of our database clearly show that important correlations could be made or further confirmed regarding vascular events, cell counts, monocyte number, etc. In spite of the short observation period patients with non-myeloid transformation were also found deserving further attention.
Keyword(s): Aspirin, Polycythemia vera, Transformation, WHO classification