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小儿双血细胞减少症/全血细胞减少症患者:三级医疗中心的病因及临床血液学特征综述

Pediatric patients with bicytopenia/pancytopenia: review of etiologies and clinico-hematological profile at a tertiary center.

作者信息

Naseem Shano, Varma Neelam, Das Reena, Ahluwalia Jasmina, Sachdeva Man Updesh Singh, Marwaha Ram Kumar

机构信息

Department of Hematology, Postgraduate Institute of Medical Education and Research, Chandigarh 160 012, India.

出版信息

Indian J Pathol Microbiol. 2011 Jan-Mar;54(1):75-80. doi: 10.4103/0377-4929.77329.

DOI:10.4103/0377-4929.77329
PMID:21393882
Abstract

BACKGROUND

The etiology of bicytopenia/pancytopenia varies widely in children, ranging from transient marrow viral suppression to marrow infiltration by fatal malignancy. Depending on the etiology, the clinical presentation can be with fever, pallor or infection. Knowing the exact etiology is important for specific treatment and prognostication.

AIMS

To evaluate the etiological and clinico-hematological profile in children with bicytopenia and pancytopenia.

MATERIALS AND METHODS

A review of bicytopenic and pancytopenic children referred for bone marrow examination from January 2007 to December 2008 was done. Detailed history, clinical examination and hematological parameters at presentation were recorded.

RESULTS AND CONCLUSION

During the study period, a total of 990 children were referred for bone marrow examination for different indications. Of these, 571 (57.7%) had either pancytopenia (17.7%) or bicytopenia (40%). Commonest form of bicytopenia was anemia and thrombocytopenia seen in 77.5% cases, followed by anemia and leukopenia in 17.3% and leukopenia and thrombocytopenia in 5.5% cases. Most common etiology was acute leukemia (66.9%) in bicytopenic children and aplastic anemia (33.8%) in pancytopenic children. Children with bicytopenia had a higher incidence of underlying malignancy (69.5% vs. 26.6%), splenomegaly (60.5% vs. 37.4%), lymphadenopathy (41.8% vs. 15.1%) and circulating blasts (64.6% vs. 20.1%) and a lower incidence of bleeding manifestations (12.1% vs. 26.6%) as compared to children with pancytopenia.

摘要

背景

儿童双血细胞减少/全血细胞减少的病因差异很大,从短暂的骨髓病毒抑制到致命恶性肿瘤的骨髓浸润。根据病因不同,临床表现可能为发热、面色苍白或感染。明确确切病因对于具体治疗和预后判断很重要。

目的

评估双血细胞减少和全血细胞减少儿童的病因及临床血液学特征。

材料与方法

对2007年1月至2008年12月因骨髓检查而转诊的双血细胞减少和全血细胞减少儿童进行回顾性研究。记录详细病史、临床表现及就诊时的血液学参数。

结果与结论

在研究期间,共有990名儿童因不同适应证接受骨髓检查。其中,571名(57.7%)患有全血细胞减少(17.7%)或双血细胞减少(40%)。双血细胞减少最常见的形式是贫血和血小板减少,见于77.5%的病例,其次是贫血和白细胞减少,占17.3%,白细胞减少和血小板减少占5.5%。双血细胞减少儿童最常见的病因是急性白血病(66.9%),全血细胞减少儿童最常见的病因是再生障碍性贫血(33.8%)。与全血细胞减少儿童相比,双血细胞减少儿童潜在恶性肿瘤的发生率更高(69.5%对26.6%)、脾肿大发生率更高(60.5%对37.4%)、淋巴结病发生率更高(41.8%对15.1%)、循环原始细胞发生率更高(64.6%对20.1%),而出血表现的发生率更低(12.1%对26.6%)。

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