Telomere length of various blood and bone marrow cells in patients with aplastic anemia

Q4 Medicine

Oncogematologiya Pub Date : 2023-09-12 DOI:10.17650/1818-8346-2023-18-3-57-64

A. V. Luchkin, E. A. Mikhailova, I. V. Galtseva, Z. T. Fidarova, A. V. Abramova, Yu. O. Davydova, N. M. Kapranov, K. A. Nikiforova, S. M. Kulikov, E. N. Parovichnikova

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引用次数: 0

Abstract

Background. Aplastic anemia proceeds with bone marrow failure and is associated with immunological suppression of normal blood stem cells’ proliferation, which lead to bone marrow aplasia. autoimmune aggression and internal defects of blood stem cell that cause abnormal hematopoiesis are being actively studied. An important role in the pathogenesis of the aplastic anemia is played by instability of telomere length (TL). determination of the initial TL makes it possible to clearly differentiate between the aplastic anemia and dyskeratosis congenita. also, it helps to identify the group of patients with short telomeres for prediction of therapy response. Aim. To investigation the TL of various blood and bone marrow cells in patients with aplastic anemia before treatment. Materials and methods. The group of patients with aplastic anemia was investigated (n = 45). blood donors (n = 32) and bone marrow donors (n = 10) of different ages were included in the reference group. adult patients with dyskeratosis congenita (n = 5) were included in the comparison group. Relative and absolute tl was identified in peripheral blood and bone marrow mononuclear cells, monocytes, lymphocytes by flow-FISH technique (combination of flow cytometry and fluorescence in situ hybridization). Results. Relative and absolute TL was comparable in different blood and bone marrow cells in patients with aplastic anemia before treatment. TL in peripheral blood and bone marrow mononuclear cells wasn’t significantly differed in groups of patients with aplastic anemia and healthy donors. Telomeres in patients with dyskeratosis congenita were identified as “ultrashort” and were significantly shorter than in patients with aplastic anemia. Conclusion. Determination of TL in patients with aplastic anemia is modern examination method, which is a necessary step of differential diagnosis between aplastic anemia and dyskeratosis congenita, which is the disease from group of constitutional bone marrow aplasia. It is preferred to identify the TL in adult patients with aplastic anemia by the flow-FISH. It is necessary to investigate the TL to predict treatment response and to identify risks of developing adverse experiences, which include relapse and clonal evolution.

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再生障碍性贫血患者各种血液和骨髓细胞的端粒长度

背景。再生障碍性贫血以骨髓衰竭为进展，并与正常造血干细胞增殖的免疫抑制有关，从而导致骨髓发育不全。自身免疫攻击和造血干细胞内部缺陷导致造血功能异常的研究正在积极进行。端粒长度不稳定在再生障碍性贫血的发病机制中起着重要作用。测定初始TL可以明确区分再生障碍性贫血和先天性角化不良。此外，它还有助于识别端粒短的患者群体，以预测治疗反应。的目标。目的探讨再生障碍性贫血患者治疗前各种血液及骨髓细胞的TL。材料和方法。对再生障碍性贫血患者组进行调查(n = 45)。参照组为不同年龄的献血者(n = 32)和骨髓献血者(n = 10)。成人先天性角化不良患者(n = 5)作为对照组。采用流式细胞术和荧光原位杂交相结合的flow- fish技术，对外周血和骨髓单核细胞、单核细胞、淋巴细胞进行相对和绝对tl鉴定。结果。治疗前再生障碍性贫血患者不同血液和骨髓细胞的相对和绝对TL具有可比性。再生障碍性贫血患者与健康供者外周血、骨髓单个核细胞TL差异无统计学意义。先天性角化不良患者的端粒被鉴定为“超短”，明显短于再生障碍性贫血患者。结论。再生障碍性贫血患者TL的测定是一种现代检查方法，是鉴别再生障碍性贫血与先天性角化不良(体质性骨髓发育不全组)的必要步骤。血流- fish是鉴别成人再生障碍性贫血患者TL的首选方法。有必要对TL进行研究，以预测治疗反应，并确定发生不良经历的风险，包括复发和克隆进化。

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来源期刊

Oncogematologiya Medicine-Hematology

CiteScore

0.50

自引率

0.00%

发文量

审稿时长

10 weeks

期刊介绍： The main purpose of the Oncohematology journal is to publish up-to-date information on clinical, experimental and fundamental scientific research, diagnostics and treatment options, as well as other materials on all relevant issues in oncohematology. The journal’s objectives are to inform various specialists who provide advisory and therapeutic assistance to patients with oncohematological diseases about current advances, including the latest methods for the diagnosis and treatment of malignant blood diseases. The journal is an interdisciplinary scientific publication uniting doctors of various specialties ‒ hematologists, oncologists, surgeons, radiation therapists, intensive care specialist, pathologists, etc. ‒ to form an interdisciplinary therapy approach in order to improve the treatment efficacy of patients with hematological malignancies.