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  • 1 Szent Borbála Kórház, Tatabánya
  • 2 Debreceni Egyetem, Általános Orvostudományi Kar, Debrecen
  • 3 Semmelweis Egyetem, Általános Orvostudományi Kar, Budapest
  • 4 Pázmány Péter Katolikus Egyetem, Piliscsaba (Esztergom)
  • 5 Somogy Megyei Kaposi Mór Oktató Kórház, Kaposvár, Tallián Gy. u. 20–32., 7400
Open access

Absztrakt:

Bevezetés és célkitűzés: A HUMYPRON munkacsoport által 2013-ban létrehozott Philadelphia-negatív myeloproliferativ neoplasiás betegek magyar regiszteréből a polycythaemia verás betegek adatait elemezzük. Módszer: A 15 hematológiai centrumból regisztrált 351 JAK2V617F- és exon-12-mutációpozitív polycythaemia verás beteg epidemiológiai, klinikai, diagnosztikus és kezelési jellemzőit, vascularis és transzformációs eseményeit dolgoztuk fel. A diagnózist megelőző és azt követő thromboemboliás eseményeket a Landolfi szerinti rizikófelmérés alapján értelmeztük. Eredmények: A diagnózist megelőzően 106 betegben 116, a diagnózist követő időszakban 106 betegben 152 thromboemboliás eseményt regisztráltunk. A major artériás események szignifikánsan csökkentek (p<0,0001), a minor vénás események szignifikánsan fokozódtak (p<0,0001) a diagnózist követően. A betegség transzformációját 26 esetben, a major vérzést 25 esetben észleltük. Következtetések: Online regiszterünk könnyen kezelhető, lehetővé teszi a hazai betegek jellemzőinek felmérését és az eredmények gyors kiértékelését. A Landolfi-rizikóbecslés eredményesnek bizonyult. Kérdőívünket a kiértékelést követően pontosítottuk. A diagnosztikus kritériumok, a rizikóbecslés és az ennek megfelelő kezelési ajánlás pontosabb betartása szükséges, amelyre ajánlásokat tettünk. Orv Hetil. 2017; 158(23): 901–909.

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