MIJELOPROLIFERATIVNE NEOPLAZME KOD BOLESNIKA MLAĐIH OD 40 GODINA: RETROSPEKTIVNA ANALIZA KLINIČKIH KARAKTERISTIKA
Sažetak
Uvod: Mijeloproliferativne neoplazme se obično dijagnostikuju kod bolesnika starosti oko 60 godina, međutim, u kliničkoj praksi se često sreću bolesnici mlađi < 40 godina.
Cilj: Procena kliničko-laboratorijskih karakteristika, učestalost tromboza i analiza terapijskog pristupa kod bolesnika sa MPN < 40 godina.
Metode: Retrospektivna studija obuhvatila je 84 bolesnika, dijagnostikovanih prema SZO kriterijumima, lečenih na Klinici za hematologiju UKCS od 2000 do 2024. godine.
Rezultati: Medijana starosti iznosila je 33 godine, sa većom učestalošću žena (60,7%). PV je nađen kod 61,9%, ET kod 25%, a prePMF kod 13,1% ispitanika. Mutacija JAK2V617F je detektovana je kod 46,5% bolesnika. Mikrovaskularne simptome imalo je 27,4%, svrab 14,3%, konstitucionalne simptome 7,1%, a splenomegaliju 45,2% bolesnika. Najviši hemoglobin i hematokrit su imali bolesnici sa PV (170 g/L, 50%). Broj trombocita bio je najveći kod bolesnika sa prePMF (1007x109/L), zatim kod ET (856x109/L) i PV (737,5x109/L). Raspodela prema stepenu fibroze koštane srži bile je sledeća: MF0 – 28,5%, MF1 – 54,8%, MF2 – 4,8%. Ukupno, 41,7% bolesnika imalo je bar jedan kardiovaskularni faktor rizika, najčešće pušenje (23,8%). Prethodne tromboze imalo je 16,7% bolesnika, a tromboze tokom praćenja (8,3%) imali su samo bolesnici sa PV. Aspirin je koristilo 86,9% bolesnika, dok su flebotomije primenjene kod skoro svih bolesnika sa PV. Citoreduktivnom terapijom lečeno je 43,9% bolesnika, 39,1% jednom TL, najčešće HU. Prema ELN skoru, 82,1% bolesnika pripadalo je grupi niskog rizika. Medijana praćenja bila je 72 meseca, a dva bolesnika su preminula (2,45%).
Zaključak: Većina mlađih bolesnika sa MPN pripada ELN grupi niskog rizika. Međutim zbog razvoja tromboze (25%), prisutnih tegoba, stepena trombocitoze i splenomegalije > 18 cm, skoro 1/2 prima citoreduktivnu terapiju. To ukazuje na potrebu bolje stratifikacije, kao i upotrebu drugačijih metoda za procenu rizika bolesti, kao što je NGS.
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