Philadelphia-negatív krónikus Myeloproliferativ Neoplasia Magyarországi Regiszter: Polycythaemia verás betegeink adatainak elemzése

Dombi Péter, A. Illés, J. Demeter, Homor Lajos, Simon Zsófia, Kellner Ádám, Karádi Éva, Valasinyószki Erika, M. Udvardy, Egyed Miklós

Research output: Article

Abstract

Intruduction and aim: The Hungarian National Registry for Philadelphia chromosome negative myeloproliferative neoplasms has been developed. The aim of the recent study is to assess the clinical characteristics of Hungarian patients with polycythemia vera. Method: Data of 351 JAK2V617F and exon 12 mutation positive polycythemia vera patients were collected online from 15 haematology centres reporting epidemiologic, clinical characteristics, diagnostic tools, therapeutic interventions, thromboembolic complications, disease transformations. Vascular events prior to and after diagnosis were evaluated upon the Landolfi risk assessment scale. Results: 116 thromboembolic events were reported in 106 PV patients prior to diagnosis and 152 occasions in 102 patients during follow-up. The frequency of major arterial events were significantly reduced (p<0.0001) and the minor venous events were significantly elevated (p<0.0001) after the diagnosis. Major hemorrhagic complications were found in 25 and transformation in 26 cases. Conclusions: Our registry allows to collect and evaluate the features of patients with polycythemia vera. The Landolfi risk stratification was proven to be useful. Based on evaluated data, accuracy of diagnostic criteria and compliance to risk-adapted therapeutic guidelines are needed.

Original languageHungarian
Pages (from-to)901-909
Number of pages9
JournalOrvosi Hetilap
Volume158
Issue number23
DOIs
Publication statusPublished - jún. 1 2017

Fingerprint

Polycythemia Vera
Registries
Neoplasms
Philadelphia Chromosome
Hematology
Compliance
Blood Vessels
Exons
Guidelines
Mutation
Therapeutics

Keywords

  • Cardiovascular risk factors
  • Landolfi risk stratification
  • Myeloproliferative neoplasms
  • Polycythemia vera
  • Registry

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Philadelphia-negatív krónikus Myeloproliferativ Neoplasia Magyarországi Regiszter : Polycythaemia verás betegeink adatainak elemzése. / Péter, Dombi; Illés, A.; Demeter, J.; Lajos, Homor; Zsófia, Simon; Ádám, Kellner; Éva, Karádi; Erika, Valasinyószki; Udvardy, M.; Miklós, Egyed.

In: Orvosi Hetilap, Vol. 158, No. 23, 01.06.2017, p. 901-909.

Research output: Article

Péter, Dombi ; Illés, A. ; Demeter, J. ; Lajos, Homor ; Zsófia, Simon ; Ádám, Kellner ; Éva, Karádi ; Erika, Valasinyószki ; Udvardy, M. ; Miklós, Egyed. / Philadelphia-negatív krónikus Myeloproliferativ Neoplasia Magyarországi Regiszter : Polycythaemia verás betegeink adatainak elemzése. In: Orvosi Hetilap. 2017 ; Vol. 158, No. 23. pp. 901-909.
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abstract = "Intruduction and aim: The Hungarian National Registry for Philadelphia chromosome negative myeloproliferative neoplasms has been developed. The aim of the recent study is to assess the clinical characteristics of Hungarian patients with polycythemia vera. Method: Data of 351 JAK2V617F and exon 12 mutation positive polycythemia vera patients were collected online from 15 haematology centres reporting epidemiologic, clinical characteristics, diagnostic tools, therapeutic interventions, thromboembolic complications, disease transformations. Vascular events prior to and after diagnosis were evaluated upon the Landolfi risk assessment scale. Results: 116 thromboembolic events were reported in 106 PV patients prior to diagnosis and 152 occasions in 102 patients during follow-up. The frequency of major arterial events were significantly reduced (p<0.0001) and the minor venous events were significantly elevated (p<0.0001) after the diagnosis. Major hemorrhagic complications were found in 25 and transformation in 26 cases. Conclusions: Our registry allows to collect and evaluate the features of patients with polycythemia vera. The Landolfi risk stratification was proven to be useful. Based on evaluated data, accuracy of diagnostic criteria and compliance to risk-adapted therapeutic guidelines are needed.",
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AU - Ádám, Kellner

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