Telomere dysfunction and its role in haematological cancer.
Observations in human tumours, as well as mouse models, have indicated that telomere dysfunction may be a key event driving genomic instability and disease progression in many solid tumour types. In this scenario, telomere shortening ultimately results in telomere dysfunction, fusion and genomic instability, creating the large-scale rearrangements that are characteristic of these tumours. It is now becoming apparent that this paradigm may also apply to haematological malignancies; indeed these conditions have provided some of the most convincing evidence of telomere dysfunction in any malignancy. Telomere length has been shown in several malignancies to provide clinically useful prognostic information, implicating telomere dysfunction in disease progression. In these malignancies extreme telomere shortening, telomere dysfunction and fusion have all been documented and correlate with the emergence of increased genomic complexity. Telomeres may therefore represent both a clinically useful prognostic tool and a potential target for therapeutic intervention.
Department of Haematology,School of Medicine, Cardiff University, Cardiff, UK.
Ceri H
CH
Jones
Chris
C
Pepper
Duncan M
DM
Baird
D000208:Acute Disease; D001855:Bone Marrow Diseases / Q000235:genetics; D018450:Disease Progression; D042822:Genomic Instability; D019337:Hematologic Neoplasms / Q000235:genetics*; D006801:Humans; D007938:Leukemia / Q000235:genetics; D016393:Lymphoma, B-Cell / Q000235:genetics; D009101:Multiple Myeloma / Q000235:genetics; D011379:Prognosis; D016615:Telomere / Q000502:physiology*; D059506:Telomere Shortening / Q000235:genetics