儿童全血细胞减少症354例临床分析  

Clinical analysis of 354 children with pancytopenia

作　　者：薛露[1] 王玥[1] 李春怀[1] 马翠[1] 

机构地区：[1]吉林大学第一医院小儿血液肿瘤科,长春130000

出　　处：《中国小儿血液与肿瘤杂志》2014年第1期43-46,共4页Journal of China Pediatric Blood and Cancer

摘　　要：目的 探讨引起儿童全血细胞减少的病因学及临床特征.方法 对354例在我科就诊的初发全血细胞减少症的患儿临床资料进行回顾性分析.结果 急性白血病和再生障碍性贫血为儿童全血细胞减少的主要病因,两者共占本组病例的71.2％;其次为噬血细胞综合征（9.9％）、感染（5.1％）、巨幼细胞性贫血（4.5％）以及免疫相关性全血细胞减少症（3.1％）,累积共占据了本组患儿的22.6％;其余病因比较少见,包括脾功能亢进、朗格罕细胞组织细胞增生症、神经母细胞瘤、脂质代谢异常、骨髓增生异常综合征、系统性红斑狼疮等,共占本组病例6.5％.中性粒细胞≤0.5×109/L者发热几率90％;血小板≤20×109/L者出血倾向严重,8.5％伴有内部脏器出血;血液系统疾病患者血红蛋白多数都波动在60 ～ 90 g/L,而非造血系统疾病血红蛋白＞90 g/L.结论 引起儿童全血细胞减少症的病因复杂,病种繁多,除急性白血病和再障外,噬血细胞综合征、感染、巨幼细胞性贫血是常见病因,其余少见病最初较难确诊,应引起儿科医生重视,就诊时血红蛋白水平可能对是否为血液系统疾病有提示意义.Objective To investigate the etiology and clinical features of the pancytopenia in children.Methods The clinical data of 354 children with pancytopenia were retrospectively reviewed.The diagnosis of pancytopenia was confirmed by laboratory examinations showing neutropenia,anemia,and thrombocytopenia.The etiologies and patient clinical features were presented.Results A total of 354 children were identified with the diagnosis of pancytopenia.Acute leukemia was the most common cause of pancytopenia （40.11%）,followed by aplastic anemia （31.07%）.Hemophagocytic syndrome （9.89%）,infectious （5.06%）,megaloblastic anemia （4.52%） and immuno-related pancytopenia （3.10%） were the more common causes,which occupied 22.51% in all patients.The other rare causes occupied 6.49% including MDS,SLE,hypersplenism et al.High fever rate （90.2%） was observed in the patients with lower neutrophils （≤0.5 × 109/L）.It has been showed that patients with lower platelets （≤20 × 109/L） had serious bleeding tendency,about 8.50% of patients with organ bleeding.The hemoglobin in patients with hemopoietic disorders mostly fluctuate in 60-90g/L.However,the hemoglobin in the patients with non-hemopoietic disorders was more than 90 g/L.Conclusion Pancytopenia in children can be caused by a wide variety of etiologies,which will lead to a diagnostic dilemma.These etiologies include hematological and non-hematological diseases.Acute leukemia and aplastic anemia are the most common etiologies.The other diseases are also important etiologies.The levels of hemoglobin are lower in patients with hemopoietic disorders than in patients with non-hematological diseases.

关 键 词：全血细胞减少 儿童 鉴别诊断 免疫 血液 

分 类 号：R725.5[医药卫生—儿科]