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Novel strategies for the treatment of myelofibrosis driven by recent advances in understanding the role of the microenvironment in its etiology [version 1; peer review: 3 approved]
- Source :
- F1000Research. 8:F1000 Faculty Rev-1662
- Publication Year :
- 2019
- Publisher :
- London, UK: F1000 Research Limited, 2019.
-
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 treatment that can modify the natural history of the disease. Therefore, the cure of myelofibrosis remains an unmet clinical need. However, the excitement raised by the discovery of the genetic lesions has inspired additional studies aimed at elucidating the mechanisms driving these neoplasms towards their final stage. These studies have generated the feeling that the cure of myelofibrosis will require targeting both the malignant stem cell clone and its supportive microenvironment. We will summarize here some of the biochemical alterations recently identified in MPNs and the novel therapeutic approaches currently under investigation inspired by these discoveries.
Details
- ISSN :
- 20461402
- Volume :
- 8
- Database :
- F1000Research
- Journal :
- F1000Research
- Notes :
- Editorial Note on the Review Process F1000 Faculty Reviews are commissioned from members of the prestigious F1000 Faculty and are edited as a service to readers. In order to make these reviews as comprehensive and accessible as possible, the referees provide input before publication and only the final, revised version is published. The referees who approved the final version are listed with their names and affiliations but without their reports on earlier versions (any comments will already have been addressed in the published version). The referees who approved this article are: Olatoyosi Odenike, Section of Hematology Oncology, Department of Medicine, University of Chicago Comprehensive Cancer Center, The University of Chicago Medicine, Chicago, IL, USA No competing interests were disclosed. Alessandro Vannucchi, Department of Hematology, Center of Research and Innovation of Myeloproliferative Neoplasms, University of Florence, Florence, Italy No competing interests were disclosed. Giovanni Martinelli, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST) IRCCS, Meldola, Italy No competing interests were disclosed. Maria Teresa Bochicchio, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST) IRCCS, Meldola, Italy No competing interests were disclosed., , [version 1; peer review: 3 approved]
- Publication Type :
- Academic Journal
- Accession number :
- edsfor.10.12688.f1000research.18581.1
- Document Type :
- review
- Full Text :
- https://doi.org/10.12688/f1000research.18581.1