Makroglobulinemia Waldenströma — diagnostyka i leczenie
Streszczenie
Makroglobulinemię Waldenströma (WM), według klasyfikacji Światowej Organizacji Zdrowia z 2017 roku, definiuje się jako współwystępowanie chłoniaka limfoplazmocytowego zajmującego szpik kostny z gammapatią monoklonalną klasy IgM niezależnie od stężenia białka monoklonalnego. Jest to rzadka choroba limfoproliferacyjna o charakterystycznych cechach klinicznych. Charakterystyka diagnostyczna w WM uległa istotnej zmianie wraz z odkryciem dwóch markerów molekularnych: MYD88 i CXCR4. Status mutacyjny tych markerów wpływa zarówno na obraz kliniczny i rokowanie, jak i wykazał implikacje terapeutyczne. Wybór leczenia w WM ściśle zależy od wieku pacjenta, ryzyka wystąpienia neuropatii związanej z leczeniem, ryzyka immunosupresji czy wtórnych nowotworów złośliwych. Krajobraz terapeutyczny poszerzył się w ostatnich latach, a zatwierdzenie ibrutynibu oraz ostatnio zanubrutynibu stanowi istotny krok naprzód w kierunku bardziej skutecznej terapii tej choroby.
Słowa kluczowe: makroglobulinemia Waldenströmachłoniak limfoplazmocytowybiałko monoklonalne klasy IgMinhibitor BTKibrutinibzanubrutynibrytuksymab
Referencje
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