High telomerase activity in granulocytes from clonal polycythemia vera and essential thrombocythemia.

@article{Ferraris2005HighTA,
  title={High telomerase activity in granulocytes from clonal polycythemia vera and essential thrombocythemia.},
  author={Anna Maria Ferraris and Rosa Mangerini and Natalija Pujic and Omar Racchi and Davide Rapezzi and Andrea Gallamini and Salvatore Casciaro and Gian Franco Gaetani},
  journal={Blood},
  year={2005},
  volume={105 5},
  pages={
          2138-40
        }
}
Essential thrombocythemia (ET) and polycythemia vera (PV) are chronic myeloproliferative disorders that share the involvement of a multipotent progenitor cell and dominance of the transformed clone over normal hematopoiesis. On the other hand, the heterogeneity of these diseases with respect to clonal development from a common progenitor has been well established. To identify useful prognostic indicators, we analyzed telomerase activity (TA), a known marker of neoplastic proliferation, in… 
Clonal granulocytes in polycythaemia vera and essential thrombocythaemia have shortened telomeres
The purpose of this study was to evaluate telomere length in peripheral blood granulocytes and mononuclear cells collected from 22 women with polycythaemia vera (PV) and essential thrombocythaemia
Polycythemia vera: new clinicopathologic perspectives.
TLDR
Modified clinical, histologic, and laboratory criteria have clarified the diagnosis of Polycythemia vera and continuing studies on the recently discovered JAK2V617F gene mutation may significantly improve the understanding of PV pathogenesis and facilitate its medical management.
New Clinicopathologic Perspectives
TLDR
Modified clinical, histologic, and laboratory criteria have clarified the diagnosis of PV and continuing studies on the recently discovered JAK2V617F gene mutation may significantly improve the understanding of PV pathogenesis and facilitate its medical management.
The expression levels of telomerase catalytic subunit hTERT and oncogenic MYC in essential thrombocythemia are affected by the molecular subtype
TLDR
The functional link between MYC and hTERT seems to be impaired depending on the molecular ET subtype, which in turn may have implications on the phenotype and course of the disease.
Telomere length is severely and similarly reduced in JAK2V617F-positive and -negative myeloproliferative neoplasms
Myeloproliferative neoplasms (MPNs) are clonal stem cell disorders characterized by chronic proliferation of hematopoietic progenitors. We studied the telomere length (TL) of 335 MPN patients and 93
The importance of the telomere and telomerase system in hematological malignancies
TLDR
The biological, diagnostic and prognostic value of telomere/telomerase biology is discussed, as well as its potential future applications in cancer therapeutics.
Telomere length dynamics in normal hematopoiesis and in disease states characterized by increased stem cell turnover
TLDR
Data from congenital disorders, like dyskeratosis congenita (DKC), suggest that disturbed telomere maintenance may play a role for replicative exhaustion of the HSC pool in vivo, and data from normal steady-state hematopoiesis provided the basis for follow-up studies in model scenarios characterized by increased HSC turnover.
Telomere and telomerase in hematologicalmalignancies.
TLDR
The importance of clinicopathologic significance of telomerase in cancer and the details of the mechanisms regulating its activity remain to be clarified.
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References

SHOWING 1-10 OF 28 REFERENCES
Clonality in myeloproliferative disorders: analysis by means of the polymerase chain reaction.
TLDR
The myeloproliferative syndromes are acquired disorders of hematopoiesis that provide insights into the transition from somatic cell mutation to neoplasia and the PCR technique is used to demonstrate heterogeneity of lineage involvement in patients with PCV.
Clonal analysis of haemopoietic cells in essential thrombocythaemia
TLDR
The findings confirm the heterogeneity of haematological abnormalities in ET patients and the potential utility of a multifaceted laboratory approach to investigate these patients.
Discrimination of polycythemias and thrombocytoses by novel, simple, accurate clonality assays and comparison with PRV-1 expression and BFU-E response to erythropoietin.
TLDR
It is shown that both genes are subject to X-chromosome inactivation and are polymorphic in all major US ethnic groups and are useful in the diagnosis and follow-up of polycythemic conditions and disorders with increased platelet levels.
Endogenous erythroid and megakaryocytic circulating progenitors, HUMARA clonality assay, and PRV-1 expression are useful tools for diagnosis of polycythemia vera and essential thrombocythemia.
TLDR
A strong correlation between polycythemia vera and essential thrombocythemia and a possible diagnostic value of some biologic markers in chronic myeloproliferative disorders is described.
Heterogeneity of clonal development in chronic myeloproliferative disorders
TLDR
A striking heterogeneity of clonal development was found in PMN from patients with other MPD, with up to 50% of them expressing a polyclonal pattern of X inactivation.
Predictive values of X-chromosome inactivation patterns and clinicohematologic parameters for vascular complications in female patients with essential thrombocythemia.
TLDR
The results suggest that older patients who have clonal XCIPs or hypertension are at increased risk for thrombosis and should be monitored closely for this complication.
Clonal hematopoiesis in familial polycythemia vera suggests the involvement of multiple mutational events in the early pathogenesis of the disease.
TLDR
The finding of erythropoietin-independent erythroid progenitors in healthy family members indicated the presence of the PV stem cell clone in their hematopoiesis, which supports the hypothesis of multiple genetic defects involved in the early pathogenesis of PV.
Quantification of PRV-1 mRNA distinguishes polycythemia vera from secondary erythrocytosis.
TLDR
A quantitative reverse transcriptase-polymerase chain reaction (RT-PCR) assay for the measurement of PRV-1 mRNA levels is reported, which has a very high sensitivity and specificity for the diagnosis of PV in patients with erythrocytosis.
A large proportion of patients with a diagnosis of essential thrombocythemia do not have a clonal disorder and may be at lower risk of thrombotic complications.
TLDR
The results suggest that ET is a heterogeneous disorder, and the clinical significance of clonality status warrants investigation in a larger study.
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