儿童全血细胞减少的临床特点和病因诊断

Clinical features and etiological spectrum in children with pancytopenia

摘要:

目的：探讨儿童全血细胞减少的临床特点及常见病因，以提高临床诊断水平。方法：收集2003年9月至2010年1月收治的174例全血细胞减少初治患儿的临床资料，对其临床表现、实验室检查、诊断结果进行分析。结果：患儿就诊时主要表现为面色苍白147例（84.5%），出血87例（50.0%），发热41例（23.6%）。血常规主要表现为轻中度贫血、血小板重度减少、白细胞轻度减少。174例患儿中由造血系统疾病引起的全血细胞减少有155例（89.1%）,非造血系统疾病（病毒感染、系统性红斑狼疮、脾功能亢进、神经母细胞瘤）6例（3.4%），13例（7.5%）原因不明。再生障碍性贫血为最常见的病因（91例，52.3%），其次为骨髓增生异常综合征（37例，21.3%）、急性白血病等血液系统肿瘤（11例，6.3%）、噬血细胞综合征（6例，3.4%）。结论：儿童全血细胞减少以贫血、出血、发热为主要临床表现，病因以再生障碍性贫血最常见，骨髓增生异常综合征、急性白血病等血液系统肿瘤、噬血细胞综合征等亦为常见病因。

Abstract:

OBJECTIVE: To study the clinical features and etiological spectrum of pancytopenia in children. METHODS: The clinical data of 174 children with pancytopenia between September 2003 and January 2010 were retrospectively reviewed. RESULTS: Pale face was the most common clinical manifestation (147 cases, 84.5%), followed by bleeding (87 cases, 50.0%) and fever (41 cases, 23.6%). Mild to moderate anemia, severe thrombocytopenia and mild leucopenia were common in complete blood count. Of the 174 children, pancytopenia was attributed to hematopoietic system diseases in 155 cases (89.1%) and non-hematopoietic system diseases (virus infections, systemic lupus erythematosus, hypersplenism and neuroblastoma) in 6 cases (3.4%). Aplastic anemia (91 cases, 52.3%) was the most common cause of pancytopenia, followed by myelodysplastic syndrome (37 cases, 21.3%), acute leukemia and other hematological tumours (11 cases, 6.3%) and hemophagocytic syndrome (6 cases, 3.4%). The cause of pancytopenia was not identified in 13 cases (7.5%). CONCLUSIONS: Anemia, bleeding and fever are the main clinical manifestations of pancytopenia in children. Pancytopenia is mostly caused by aplastic anemia in children. Myelodysplastic syndrome, hematological tumours and hemophagocytic syndrome are also the common causes.