الفهرس | Only 14 pages are availabe for public view |
Abstract Background and Aim of The Work: Bone marrow failure syndromes (BMFS) includes inherited and acquired conditions. Inherited bone marrow failure includes a number of syndromes; with Fanconi anemia (FA) being the most common one of them. Telomeres are eroded with cell division, but in hematopoietic stem cell, maintenance of their length is mediated by telomerase. Short telomeres can result in instability of cell function where diseases occur. Bone Marrow Failure might be developed due to low telomerase activity or short telomeres. Our study aims to use and evaluate the utility of real time quantitative-polymerase chain reaction (RQ-PCR) in measuring the relative telomere length (RTL). Its correlation with the biochemistry and the clinicopathological data of the Egyptian patients with bone marrow failure (BMF) either acquired or inherited, and its significance in the diagnosis and prognosis of these patients were investigated. Materials and Methods: The study includes 3 groups: A group of congenital BMF (29 patients), a group of acquired BMF (10 patients) and a third control group (15 cases). The relative telomere length is evaluated for them using RT-qPCR. Results: We have found that there is a significant difference in relative telomere length between congenital group and controls (p=0.001), also a significant difference between acquired group and controls (p= 0.029). However, there is no significant difference between congenital and acquired groups (p= 0.479). There is no significant correlation between the telomere length and the overall survival or prognosis of the patients of BMFS. Conclusion: RTL is significantly altered in Egyptian patients with BMF whether being inherited or acquired conditions compared to control group. We found an inverse correlation between RTL and lymphocytes, while the RTL has a positive correlation with red blood cells (RBCs). In our study, the degree of telomere shortening does not have relation with patient`s prognosis or overall survival |