PROGRESIJA BOLESTI KOD PACIJENATA SA PRIMARNOM MIJELOFIBROZOM NISKOG RIZIKA – PRIKAZ SLUČAJA
Sažetak
Uvod: Medijana preživljavanja (OS) pacijenata sa PMF-om niskog rizika je preko 15 godina, ali prema „MOST” prospektivnoj studiji, progresiju bolesti ima skoro 60% ovih pacijenata.
Cilj: Predstaviti tok bolesti i lečenje pacijenata sa PMF-om niskog rizika.
Prikaz slučajeva: Svi pacijenti su imali utvrđenu PMF, IPSS niskog rizika i normalnu početnu citogenetiku.
Slučaj 1: Pacijent, muškarac starosti 61 godina, primljen je u maju 2016. godine sa Tr 772x109/L, LDH 566 U/L, bez splenomegalije, JAK2V617F+. Prvobitno je lečen hidroksiureom (HU) od 2016. do 2020. godine. Od 2021. godine uveden je ruksolitinibom zbog razvoja splenomegalije i leukocitoze, uz progresiju bolesti 18 meseci kasnije (slezina 26 cm, Plt 20x109/L, 10% blasta u srži, složen kariotip: -5, del 7q, mar+). Uveden je azacitidin, ali zbog sepse dolazi do smrtnog ishoda u oktobru 2022. godine. OS je 66 meseci.
Slučaj 2: Pacijentkinja, žena starosti 47 godina, primljena je u julu 2011. godine sa Tr 899x109/L, LDH 899 U/L, slezinom 15x7 cm, JAk2V617F+, ASXL+. Inicijalno je lečena sa HU, a od oktobra 2013. godine ruxolitinibom, zbog progresije splenomegalije (slezina 19,3 cm, LDH 1881 U/L), uz potonju normalizaciju veličine slezine, broja Tr i LDH. Pacijentkinja je u remisiji. OS je 126 meseci.
Slučaj 3: Pacijent, muškarac starosti 64 godine, primljen je u maju 2012. godine sa Tr 1457x109/L, LDH 631 U/L, graničnom splenomegalijom, JAk2V617F-, MPL+, SRSF2+, U2AF1+, ASXL1+. Inicijalno je lečen sa HU (maj 2012. godine), a od aprila 2019. godine danazolom zbog pojave transfuzione zavisnosti. Zbog srčane insuficijencije, smrtni ishod je nastupio u februaru 2020. godine. OS je 96 meseci.
Zaključak: Neki pacijenti sa PMF-om niskog rizika imaju progresiju bolesti, a buduća istraživanja će pokazati da li rana NGS analiza non-driver mutacija i rano uvođenje terapije doprinose promeni toka bolesti.
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