作者: Ruben A. Mesa
DOI: 10.1007/978-1-60761-266-7_9
关键词: Medicine 、 Transplantation 、 Induction chemotherapy 、 Acute leukemia 、 Stem cell 、 Oncology 、 Constitutional symptoms 、 Essential thrombocythemia 、 Internal medicine 、 Myelofibrosis 、 Polycythemia 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.