Blastic Transformation of Classic Myeloproliferative Neoplasms

作者: Ruben A. Mesa

DOI: 10.1007/978-1-60761-266-7_9

关键词: MedicineTransplantationInduction chemotherapyAcute leukemiaStem cellOncologyConstitutional symptomsEssential thrombocythemiaInternal medicineMyelofibrosisPolycythemia vera

摘要: The BCR-ABL negative myeloproliferative neoplasms (MPNs) of essential thrombocythemia, polycythemia vera, and primary myelofibrosis have an increasing predisposition over the course a patient’s illness to transform overt acute leukemia what is referred as MPN-Blast Phase (MPN-BP). Current therapies for MPNs are limited, no therapy other than allogeneic stem cell transplant has clearly altered natural history these neoplasms. Pathogenetic mechanisms which lead MPN progressing MPN-BP incompletely understood, but seem correlate with accumulation additional karyotypic abnormalities opposed increases in MPN-associated molecular lesion burden (such JAK2V617F). development heralded by worsening cytopenias, constitutional symptoms, very poor survival despite therapeutic intervention. Risk factors developing include both features at diagnosis increased peripheral blood blasts, abnormalities, thrombocytopenia), well exposure established agents enhance leukemogenesis (i.e., P-32 alkylators). Salvage induction chemotherapy followed possible, successful option small minority patients. Multiple avenues investigation ongoing treat, or prevent, including early transplantation, hypomethylating agents, JAK2 inhibition. An improved understanding pathogenetic underpinings necessary if more effective targeted be developed.

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