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Overview of the Mutational Landscape in Primary Myelofibrosis and Advances in Novel Therapeutics.
Asian Pac J Cancer Prev 2019; 20(6):1691-1699AP

Abstract

Primary Myelofibrosis is a BCR-ABL negative myeloproliferative neoplasm with a variety of hematological presentations, including thrombosis, bleeding diathesis and marrow fibrosis. It is estimated to have an incidence of 1.5 per 100,000 people each year. Although JAK2 or MPL mutations are seen in PMF, several other mutations have recently been documented, including mutations in CALR, epigenetic regulators like TET, ASXL1, and 13q deletions. The identification of these mutations has improved the ability to develop novel treatment options. These include JAK inhibitors like ruxolitinib, heat shock protein-90 inhibitors like ganetespib, histone deacetylase inhibitors including panobinostat, pracinostat, vorinostat and givinostat, hypomethylating agents like decitabine, hedgehog inhibitors like glasdegib, PI3K, AKT and mTOR inhibitors like everolimus as well as telomerase inhibitors like imtelstat. Research on novel therapeutic options is being actively pursued in order to expand treatment options for primary myelofibrosis however currently, there is no curative therapy other than allogenic hematopoietic stem cell transplantation (ASCT) which is possible in select patients.

Authors+Show Affiliations

Ziauddin Hospital, Karachi, Pakistan. Email: jaleedahmedgi16@hotmail.comAga Khan University Hospital, Karachi, Pakistan.Department of Internal Medicine, West Virginia University, Morgantown, WV, USA.Department of Oncology, Aga Khan University Hospital, Karachi, Pakistan.Ziauddin Hospital, Karachi, Pakistan. Email: jaleedahmedgi16@hotmail.comDepartment of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

31244289

Citation

Gilani, Jaleed Ahmed, et al. "Overview of the Mutational Landscape in Primary Myelofibrosis and Advances in Novel Therapeutics." Asian Pacific Journal of Cancer Prevention : APJCP, vol. 20, no. 6, 2019, pp. 1691-1699.
Gilani JA, Ashfaq MA, Mansoor AE, et al. Overview of the Mutational Landscape in Primary Myelofibrosis and Advances in Novel Therapeutics. Asian Pac J Cancer Prev. 2019;20(6):1691-1699.
Gilani, J. A., Ashfaq, M. A., Mansoor, A. E., Abdul Jabbar, A., Siddiqui, T., & Khan, M. (2019). Overview of the Mutational Landscape in Primary Myelofibrosis and Advances in Novel Therapeutics. Asian Pacific Journal of Cancer Prevention : APJCP, 20(6), pp. 1691-1699. doi:10.31557/APJCP.2019.20.6.1691.
Gilani JA, et al. Overview of the Mutational Landscape in Primary Myelofibrosis and Advances in Novel Therapeutics. Asian Pac J Cancer Prev. 2019 06 1;20(6):1691-1699. PubMed PMID: 31244289.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Overview of the Mutational Landscape in Primary Myelofibrosis and Advances in Novel Therapeutics. AU - Gilani,Jaleed Ahmed, AU - Ashfaq,Muhammad Areeb, AU - Mansoor,Armaghan-E-Rehman, AU - Abdul Jabbar,Adnan, AU - Siddiqui,Tariq, AU - Khan,Maliha, Y1 - 2019/06/01/ PY - 2018/08/13/received PY - 2019/6/28/entrez PY - 2019/6/28/pubmed PY - 2019/6/28/medline KW - Thrombocytosis KW - myeloproliferative disorders KW - primary myelofibrosis SP - 1691 EP - 1699 JF - Asian Pacific journal of cancer prevention : APJCP JO - Asian Pac. J. Cancer Prev. VL - 20 IS - 6 N2 - Primary Myelofibrosis is a BCR-ABL negative myeloproliferative neoplasm with a variety of hematological presentations, including thrombosis, bleeding diathesis and marrow fibrosis. It is estimated to have an incidence of 1.5 per 100,000 people each year. Although JAK2 or MPL mutations are seen in PMF, several other mutations have recently been documented, including mutations in CALR, epigenetic regulators like TET, ASXL1, and 13q deletions. The identification of these mutations has improved the ability to develop novel treatment options. These include JAK inhibitors like ruxolitinib, heat shock protein-90 inhibitors like ganetespib, histone deacetylase inhibitors including panobinostat, pracinostat, vorinostat and givinostat, hypomethylating agents like decitabine, hedgehog inhibitors like glasdegib, PI3K, AKT and mTOR inhibitors like everolimus as well as telomerase inhibitors like imtelstat. Research on novel therapeutic options is being actively pursued in order to expand treatment options for primary myelofibrosis however currently, there is no curative therapy other than allogenic hematopoietic stem cell transplantation (ASCT) which is possible in select patients. SN - 2476-762X UR - https://www.unboundmedicine.com/medline/citation/31244289/Overview_of_the_Mutational_Landscape_in_Primary_Myelofibrosis_and_Advances_in_Novel_Therapeutics L2 - http://journal.waocp.org/?sid=Entrez:PubMed&id=pmid:31244289&key=2019.20.6.1691 DB - PRIME DP - Unbound Medicine ER -