TY - JOUR
T1 - Novel insights into the biology and treatment of chronic myeloproliferative neoplasms
AU - Mughal, Tariq I.
AU - Barbui, Tiziano
AU - Abdel-Wahab, Omar
AU - Kralovics, Robert
AU - Jamieson, Catriona
AU - Kvasnicka, Hans Michael
AU - Mullaly, Ann
AU - Rampal, Raajit
AU - Mesa, Ruben
AU - Kiladjian, Jean Jacques
AU - Deininger, Michael
AU - Prchal, Josef
AU - Hehlmann, Rüdiger
AU - Saglio, Giuseppe
AU - Van Etten, Richard A.
N1 - Publisher Copyright:
© 2014 Informa UK, Ltd.
PY - 2015/7/1
Y1 - 2015/7/1
N2 - Myeloproliferative neoplasms (MPNs) are clonal disorders of hematopoiesis characterized by a high frequency of genetic alterations, and include chronic myeloid leukemia (CML) and the BCR-ABL1-negative MPNs. Herein we summarize recent advances and controversies in our understanding of the biology and therapy of these disorders, as discussed at the 8th post-American Society of Hematology CML-MPN workshop. The principal areas addressed include the breakthrough discovery of CALR mutations in patients with JAK2/MPL wild type MPN, candidate therapies based on novel genetic findings in leukemic transformation and new therapeutic targets in MPNs, and an appraisal of bone marrow histopathology in MPNs with a focus on the potential new clinical entity of "masked" polycythemia vera. An update on clinical trials of Janus kinase (JAK) inhibitors is presented as well as current understanding regarding the definitions and mechanisms of resistance to JAK inhibitors, and updated information on the safety and efficacy of discontinuation of tyrosine kinase inhibitors in patients with CML.
AB - Myeloproliferative neoplasms (MPNs) are clonal disorders of hematopoiesis characterized by a high frequency of genetic alterations, and include chronic myeloid leukemia (CML) and the BCR-ABL1-negative MPNs. Herein we summarize recent advances and controversies in our understanding of the biology and therapy of these disorders, as discussed at the 8th post-American Society of Hematology CML-MPN workshop. The principal areas addressed include the breakthrough discovery of CALR mutations in patients with JAK2/MPL wild type MPN, candidate therapies based on novel genetic findings in leukemic transformation and new therapeutic targets in MPNs, and an appraisal of bone marrow histopathology in MPNs with a focus on the potential new clinical entity of "masked" polycythemia vera. An update on clinical trials of Janus kinase (JAK) inhibitors is presented as well as current understanding regarding the definitions and mechanisms of resistance to JAK inhibitors, and updated information on the safety and efficacy of discontinuation of tyrosine kinase inhibitors in patients with CML.
KW - Molecular genetics
KW - Myeloid leukemias and dysplasias
KW - Myeloproliferative disorders
KW - Prognostication
KW - Signaling therapies
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U2 - 10.3109/10428194.2014.974594
DO - 10.3109/10428194.2014.974594
M3 - Review article
C2 - 25330439
AN - SCOPUS:84938149109
SN - 1042-8194
VL - 56
SP - 1938
EP - 1948
JO - Leukemia and Lymphoma
JF - Leukemia and Lymphoma
IS - 7
ER -