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Tom 3, Nr 1-2 (2023)
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Wyświetlenia strony 358
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Eksport do Mediów Społecznościowych

Eksport do Mediów Społecznościowych

Jak zapobiegać zespołowi niedrożności zatokowej wątroby/chorobie zarostowej żył wątrobowych?

Patrycja Mensah-Glanowska1, Paulina Pyrcak1, Anna Czyż2
Hematologia - Edukacja 2023;3(1-2):22-31.

Streszczenie

Zespół niedrożności zatokowej wątroby/choroba zarostowa żył wątrobowych (SOS/VOD) to zagrażające życiu powikłanie po transplantacji krwiotwórczych komórek macierzystych (HSCT). Klinicznie manifestuje się zespołem objawów, wśród których najważniejszymi są: żółtaczka, zwiększenie masy ciała, wodobrzusze i bolesne powiększenie wątroby. U pacjentów z rozwijającą się ciężką postacią SOS/VOD dochodzi do wystąpienia poważnych powikłań w postaci niewydolności oddechowej, uszkodzenia funkcji nerek i niewydolności sercowo-naczyniowej i, ostatecznie, niewydolności wielonarządowej, której pojawienie się wiąże się ze śmiertelnością wynoszącą ponad 80%. Częstość występowania SOS/VOD po HSCT szacuje się na 5–15%, ale istotnie zależy od populacji chorych — wieku i wskazania do transplantacji, intensywności kondycjonowania oraz kryteriów rozpoznania. Największa częstość tego powikłania występowała w dekadach, w których w transplantacji szpiku dominowało kondycjonowanie mieloablacyjne oparte na doustnej postaci busulfanu. Wprowadzenia do leczenia ostrych białaczek koniugatów przeciwciał monoklonalnych z ozogamycyną sprawiło, że zainteresowanie profilaktyką i leczeniem SOS/VOD ponownie wzrosło ze względu na zwiększoną częstość występowania tego powikłania u chorych po transplantacją poprzedzonej stosowaniem wymienionych wyżej nowych leków. W niniejszej pracy przedstawiono przegląd współczesnej wiedzy na temat kryteriów rozpoznania, czynników ryzyka, biomarkerów, technik diagnostycznych oraz profilaktyki farmakologicznej i niefarmakologicznej SOS/VOD.

Słowa kluczowe: zespół niedrożności zatokowej wątroby/choroba zarostowa żył wątrobowychkryteria rozpoznaniaczynniki ryzykaprofilaktyka

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