儿童全血细胞减少症的临床特征及病因分析(1)
摘要：目的 分析儿童全血细胞减少症的病因谱，探讨儿童全血细胞减少症的临床特点，以提高临床对该病的诊断。方法 选择2013年1月～2019年12月我院住院的119例全血细胞减少症患者的临床资料，分析其外周血细胞计数检查、血涂片人工显微镜检查、骨髓活组织检查、生化检查、流式细胞检测、病原学检查、影像学检查及基因检查等资料，总结其临床特征及病因。结果 全血细胞减少症最常见的临床特征是苍白、乏力、感染、出血;患者血红蛋白水平为(63.00±16.00)g/L，白細胞计数(2.45±0.57)×109/L，中性粒细胞计数(0.70±0.32)×109/L，血小板计数(43.58±35.24)×109/L，网织红细胞计数(0.043±0.038)×1012/L;44例(36.97%)全血细胞减少症患者为低增生性骨髓，75例(63.03%)为非低增生性骨髓，两者血细胞减少患者的比例比较，差异有统计学意义(P<0.05)。病因学分析显示，再生障碍性贫血(25.21%)是全血细胞减少症最常见的原因，其次是急性白血病(22.69%)及噬血细胞性淋巴组织细胞增生症(9.24%)。结论 全血细胞减少症是临床较常见的血液系统综合征，病因学分类复杂，最常见的病因仍为再生障碍性贫血，但由于诊断技术的进步，基因检测成为先天性骨髓衰竭及遗传代谢病等疾病确诊重要的检测手段，大大丰富了病因谱系，提高了明确诊断率。
关键词：全血细胞减少症;病因;临床特征;再生障碍性贫血;儿童
中图分类号：R725.5 文献标识码：A DOI：10.3969/j.issn.1006-1959.2020.13.027
文章编号：1006-1959(2020)13-0099-05
Clinical Characteristics and Etiological Analysis of Childhood Pancytopenia
CHEN Hui，LIU Li，CHEN Sen
(Department of Hematology，Tianjin Children's Hospital，Tianjin 300400，China)
Abstract：Objective To analyze the etiology spectrum of children's pancytopenia and explore the clinical features of children's pancytopenia to improve the clinical diagnosis of the disease.Methods The clinical data of 119 patients with pancytopenia hospitalized in our hospital from January 2013 to December 2019 were selected， and their peripheral blood cell count examination， blood smear manual microscopy， bone marrow biopsy， biochemical examination， and flow cytometry were analyzed. Cell detection， etiological examination， imaging examination and genetic examination and other data to summarize its clinical characteristics and causes.Results The most common clinical features of pancytopenia are paleness， fatigue， infection， and bleeding; the patient's hemoglobin level is (63.00±16.00) g/L ， White blood cell count (2.45±0.57)×109/L， neutrophil count (0.70±0.32)×109/L， platelet count (43.58±35.24)×109/L， reticulocyte count (0.043±0.038)×1012/L; 44 cases (36.97%) of patients with pancytopenia had hypoproliferative bone marrow and 75 cases (63.03%) had non-hyperproliferative bone marrow. There was a statistically significant difference in the proportion of patients with cytopenia (P<0.05). Etiological analysis showed that aplastic anemia (25.21%) is the most common cause of pancytopenia， followed by acute leukemia (22.69%) and hematopoietic lymphohistiocytosis (9.24%). Conclusion Pancytopenia is a common clinical hematological syndrome. The etiology is complicated. The most common cause is still aplastic anemia. However， due to the advancement of diagnostic technology， genetic testing has become congenital bone marrow failure and genetic metabolic diseases. The important detection methods for the diagnosis of diseases have greatly enriched the etiology and improved the diagnosis rate., http://www.100md.com(陈辉 刘力 陈森)
关键词：全血细胞减少症;病因;临床特征;再生障碍性贫血;儿童
中图分类号：R725.5 文献标识码：A DOI：10.3969/j.issn.1006-1959.2020.13.027
文章编号：1006-1959(2020)13-0099-05
Clinical Characteristics and Etiological Analysis of Childhood Pancytopenia
CHEN Hui，LIU Li，CHEN Sen
(Department of Hematology，Tianjin Children's Hospital，Tianjin 300400，China)
Abstract：Objective To analyze the etiology spectrum of children's pancytopenia and explore the clinical features of children's pancytopenia to improve the clinical diagnosis of the disease.Methods The clinical data of 119 patients with pancytopenia hospitalized in our hospital from January 2013 to December 2019 were selected， and their peripheral blood cell count examination， blood smear manual microscopy， bone marrow biopsy， biochemical examination， and flow cytometry were analyzed. Cell detection， etiological examination， imaging examination and genetic examination and other data to summarize its clinical characteristics and causes.Results The most common clinical features of pancytopenia are paleness， fatigue， infection， and bleeding; the patient's hemoglobin level is (63.00±16.00) g/L ， White blood cell count (2.45±0.57)×109/L， neutrophil count (0.70±0.32)×109/L， platelet count (43.58±35.24)×109/L， reticulocyte count (0.043±0.038)×1012/L; 44 cases (36.97%) of patients with pancytopenia had hypoproliferative bone marrow and 75 cases (63.03%) had non-hyperproliferative bone marrow. There was a statistically significant difference in the proportion of patients with cytopenia (P<0.05). Etiological analysis showed that aplastic anemia (25.21%) is the most common cause of pancytopenia， followed by acute leukemia (22.69%) and hematopoietic lymphohistiocytosis (9.24%). Conclusion Pancytopenia is a common clinical hematological syndrome. The etiology is complicated. The most common cause is still aplastic anemia. However， due to the advancement of diagnostic technology， genetic testing has become congenital bone marrow failure and genetic metabolic diseases. The important detection methods for the diagnosis of diseases have greatly enriched the etiology and improved the diagnosis rate., http://www.100md.com(陈辉 刘力 陈森)