Novel strategies for the treatment of myelofibrosis driven by recent advances in understanding the role of the microenvironment in its etiology.

ABSTRACT: Myelofibrosis is the advanced stage of the Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs), characterized by systemic inflammation, hematopoietic failure in the bone marrow, and development of extramedullary hematopoiesis, mainly in the spleen. The only potentially curative therapy for this disease is hematopoietic stem cell transplantation, an option that may be offered only to those patients with a compatible donor and with an age and functional status that may face its toxicity. By contrast, with the Philadelphia-positive MPNs that can be dramatically modified by inhibitors of the novel BCR-ABL fusion-protein generated by its genetic lesion, the identification of the molecular lesions that lead to the development of myelofibrosis has not yet translated into a treatm

SUBMITTER: Eran Z 

PROVIDER: S-EPMC6758840 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature