儿科骨髓衰竭的登记和临床谱研究单中心体验

Asmaa Abd Elrahman, M. Meabed, Rehab M. Abd Elkareem, Yasmine Mohamed
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摘要

背景:骨髓衰竭是一组以骨髓不能产生足够的血细胞为特征的疾病。可分为遗传性疾病、获得性疾病和浸润性骨髓疾病。急性白血病是一种严重的骨髓衰竭,需要早期诊断和治疗。目的:研究贝绥大学附属医院骨髓衰竭的登记、临床谱,了解骨髓衰竭的严重程度。患者和方法:采用横断面研究,于2019年1月至2019年6月为期6个月,纳入贝尼-苏夫大学附属医院儿科收治的儿童年龄组(0-13岁)骨髓衰竭(单核细胞减少症、双细胞减少症或全细胞减少症)患者并进行随访。他们接受了详细的病史、全面的检查和实验室调查;全血细胞计数、网织细胞计数、骨髓抽吸和活检、病毒学研究以及相应的其他调查,如cd55和cd59的流式细胞术、葡萄糖脑苷酶测定、染色体断裂分析和成像。结果:门诊22000例,住院1700例,6个月内诊断为骨髓衰竭的患者55例,占0.23%。骨髓衰竭最常见的诊断是急性白血病(30.9%),其次是特发性再生障碍性贫血(27.3%)和范可尼贫血(21.8%)。结论:骨髓衰竭并不罕见,急性白血病是诊断骨髓衰竭的重要因素。
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Study for registry and clinical spectrum of bone marrow failure in pediatrics; single centre experience
: Background : Bone marrow failure is a group of disorders characterized by the inability of bone marrow to produce sufficient blood cells. They can be divided into inherited disorders, acquired disorders and diseases infiltrating bone marrow. Acute leukemia is a serious cause of bone marrow failure which needs early diagnosis and management . Purpose : To study for registry, and clinical spectrum of bone marrow failure in Beni-Suef university hospital to detect the magnitude of the problem . Patients and methods: It is cross sectional study over a period of six months from January 2019 to June 2019, in which patients with bone marrow failure (monocytopenia, bicytopenia or pancytopenia) from pediatric age group (0-13 years) who were admitted in the pediatric department in Beni-Suef university hospital were included and followed up. They were subjected to a detailed history, full examination and laboratory investigation; complete blood count, reticulocyte cont, bone marrow aspirate & biopsy, virology study and accordingly other investigations were done such as flow cytometry for cd55 & cd59, glucocerebrosidase enzyme assay, chromosomal breakage analysis and imaging. Results: Out of 22000 outpatients and 1700 inpatients,55 patients had diagnosed as bone marrow failure during six months duration with percentage of o.23%. The most frequent diagnosis of bone marrow failure was acute Leukemia (30.9 %), followed by Idiopathic aplastic anemia (27.3%) then Fanconi anaemia (21.8%). Conclusion: Bone marrow failure is not rare ,acute Leukemia should be considered strongly in the diagnosis of any case of bone marrow failure .
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