MORFOLOGIJA MIJELODISPLASTIČNIH/MIJELOPROLIFERATIVNIH NEOPLAZMA (MDS/MPN)

Biljana Jelić Puškarić, Gordana Kaić, Marina Pažur, Mia Šunjić Stakor, Suzana Katalenić Simon, Inga Mandac Rogulj, Slobodanka Ostojić Kolonić, Delfa Radić Krišto, Ika Kardum-Skelin

Dijagnostička kategorija mijelodisplastične/mijeloproliferativne neoplazme (MDS/MPN) obuhvaća klonske hematopoetske neoplazme koje u vrijeme postavljanja dijagnoze istodobno pokazuju klinička, laboratorijska i/ili morfološka obilježja i mijelodisplastičnog sindroma (MDS) i mijeloproliferativne neoplazme (MPN). Citopenija i displazija jedne ili više mijeloidnih loza (obilježja MDS-a) mogu se vidjeti istodobno s leukocitozom, trombocitozom i/ili organomegalijom (obilježja koja su češće povezana s MPN-om). Bolesnici s prije dijagnosticiranim MPN-om, kod kojih se razviju mijelodisplastične promjene kao posljedica evolucije bolesti ili kemoterapije, ne ubrajaju se u ovu dijagnostičku kategoriju. Prema klasifikaciji Svjetske zdravstvene organizacije (engl. World Health Organization – WHO) iz 2008. godine te njezinoj reviziji iz 2016. godine, MDS/MPN obuhvaća pet entiteta: kroničnu mijelomonocitnu leukemiju (engl. chronic myelomonocytic leukemia – CMML), juvenilnu mijelomonocitnu leukemiju (engl. juvenile myelomonocytic leukemia – JMML), atipičnu kroničnu mijeloičnu leukemiju, BCR-ABL1^- (engl. atypical chronic myeloid leukemia – aCML), MDS/MPN s prstenastim sideroblastima i trombocitozom (engl. MDS/MPN with ring sideroblasts and thrombocytosis – MDS/MPN-RS-T) i neklasificirani MDS/MPN (engl. MDS/MPN, unclassifiable – MDS/MPN, U).

Diagnostic category of MDS/MPN includes clonal hematopoietic neoplasms, which show the concomitant clinical, laboratory and/or morphologic features of both myelodysplastic syndrome (MDS) and myeloproliferative neoplasm (MPN) at the time of diagnosis. Cytopenia and dysplasia of one or more myeloid lineages (the MDS features) can be present accompanied with leukocytosis, thrombocytosis and/or organomegaly (all features more often related to MPN). Patients with a previous diagnosis of MPN who develop the myelodysplastic alterations secondary to disease evolution or chemotherapy are not assigned to this diagnostic category. According to the WHO (World Health Organization) classification from 2008 and the 2016 revision, the MDS/MPN category includes five entities as follows: CMML (chronic myelomonocytic leukemia), JMML (juvenile myelomonocytic leukemia), aCML (atypical chronic myeloid leukemia) BCR-ABL1^–, MDS/MPN-RS-T (MDS/MPN with ring sideroblasts and thrombocytosis) and MDS/MPN-U (MDS/MPN, unclassifiable).