Prechod primárnej myelofibrózy do akútnej myeloidnej leukémie: patogenéza, diagnostika a klinické súvislosti leukemickej transformácie

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Autori/ Authors:       Jaromír Tupý      Ivan Ondrášik      Miriam Tupá      Katarína Ondrášiková

In: Zdravotnícke štúdie

ISSN: 1337-723X

Ročník/ Volume: XVIII

Číslo/ Issue: 2

Strany/ Page Range: 28-35

Rok vydania/ Publication Year: 2026

Jazyk/ Language: English

Vydavateľ/ Publisher: VERBUM – vydavateľstvo Katolíckej univerzity v Ružomberku

Abstrakt/ Abstract:
Primary myelofibrosis (PMF) is a Philadelphia-chromosome-negative myeloproliferative neoplasm characterised by clonal proliferation of atypical megakaryocytes, progressive bone-marrow fibrosis, extramedullary haematopoiesis and a substantial risk of leukaemic transformation into secondary acute myeloid leukaemia (post-MF AML), which remains one of the most treatment-resistant forms of secondary leukemia, underscoring the need for early molecular risk stratification and early consideration of allogeneic hematopoietic stem cell transplantation.
This review summarises current knowledge of PMF, including its definition, aetiology, pathophysiology centred on constitutive JAK -STAT signalling driven, and classification of myeloproliferative neoplasms. Diagnostic work-up is presented in detail across morphological, histopathological, immunophenotypic, molecular-genetic and imaging modalities. The core of the review addresses the biological basis of transformation from chronic-phase PMF through an accelerated phase to overt blast-phase disease (post-MF AML), emphasising the role of highmolecular-risk mutations, clonal evolution and karyotypic instability, epigenetic dysregulation, bone-marrow niche remodelling and impaired immune surveillance. Prognostic scoring systems and their relevance to predicting transformation risk are discussed, together with the clinical and therapeutic implications of blast-phase disease.

Kľúčové slová/ Keywords:  Primary myelofibrosis. Myeloproliferative neoplasm. Leukaemic transformation. Blast phase. Acute myeloid leukaemia. Clonal evolution. Bone marrow fibrosis.

Citácia/ How to cite:
PRIEZVISKO, Meno, 2026. Progression of Primary Myelofibrosis to Acute Myeloid Leukemia: Pathogenesis, Diagnosis, and Clinical Aspects of Leukemic Transformation. Zdravotnícke štúdie. Online. Vol. XVIII, no. 2,pp. 28-35. ISSN 1337-723X. Available from: 

Toto dielo je publikované pod/ This work is licensed under a Creative Commons Attribution 4.0 International License.