title: Dynamics of telomere's length and telomerase activity in Philadelphia chromosome negative myeloproliferative neoplasms.
abstract: Telomere exhaustion and increased telomerase activity are associated with the acquisition of aggressive molecular events in a variety of haematological malignancies. In Philadelphia chromosome negative myeloproliferative neoplasms (Ph(neg)MPN's), telomere dynamics during clonal evolution of these diseases have not yet been fully elucidated. Herein we demonstrated that telomere shortening is a global phenomenon in Ph(neg)MPN's, irrespective of disease phenotype, treatment administration and JAK2V617F mutational status but the presence of additional cytogenetic abnormalities further affects them. Consistent with the above finding, TA was upregulated in CD34+ haemopoietic progenitors from almost all Ph(neg)MPN subgroups compared to healthy donors. Moreover, TL below the cut-off value of 27% could predict disease progression in Ph(neg)MPN patients (PFS at 5 years 39% vs 81%). Thus, TL emerges as a new prognostic marker in Ph(neg)MPN, reflecting probably the genetic instability of highly proliferating MPN clones.
authors.affiliation: Department of Haematology, Democritus University of Thrace, Medical School, Alexandroupolis, Greece.
authors.forename: Emmanouil
authors.initials: E
authors.lastname: Spanoudakis
authors.forename: Ioanna
authors.initials: I
authors.lastname: Bazdiara
authors.forename: Despoina
authors.initials: D
authors.lastname: Pantelidou
authors.forename: Ioannis
authors.initials: I
authors.lastname: Kotsianidis
authors.forename: Vasilios
authors.initials: V
authors.lastname: Papadopoulos
authors.forename: Dimitrios
authors.initials: D
authors.lastname: Margaritis
authors.forename: George
authors.initials: G
authors.lastname: Xanthopoulidis
authors.forename: Eleftherios
authors.initials: E
authors.lastname: Moustakidis
authors.forename: Stamatia
authors.initials: S
authors.lastname: Mantzourani
authors.forename: George
authors.initials: G
authors.lastname: Bourikas
authors.forename: Costas
authors.initials: C
authors.lastname: Tsatalas
mesh_terms: D001854:Bone Marrow Cells / Q000201:enzymology / Q000378:metabolism; D002478:Cells, Cultured; D015870:Gene Expression; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D053614:Janus Kinase 2 / Q000235:genetics; D009154:Mutation; D009196:Myeloproliferative Disorders / Q000188:drug therapy / Q000201:enzymology* / Q000235:genetics*; D010677:Philadelphia Chromosome; D011379:Prognosis; D020133:Reverse Transcriptase Polymerase Chain Reaction; D019098:Telomerase / Q000235:genetics / Q000378:metabolism*; D016615:Telomere / Q000235:genetics*
chemical_list: D053614:Janus Kinase 2; D019098:Telomerase