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高免疫球蛋白M综合征伴纯红细胞再生障碍性贫血1例 被引量：1

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摘要 高免疫球蛋白M综合征（hyper IgM syndrome，HIGM）1961年由Rosen等首次报道，是一种罕见的原发性免疫缺陷病，人群总体患病率约为百万分之一，HIGM除常伴发感染外，易合并自身免疫性疾病，如溶血性贫血、中性粒细胞减少症、血小板减少症、虹膜睫状体炎、幼年特发性关节炎、过敏性紫癜等。中国医学科学院血液病医院儿科最近收治1例HIGM患儿伴纯红细胞再生障碍性贫血，在国内外尚属首次报道。
作者 赖长城 王书春 周剑峰 彭广新 王津雨 竺晓凡 陈晓娟
机构地区 江西省儿童医院血液科 中国医学科学院、北京协和医学院血液研究所、血液病医院儿科
出处 《中国实用儿科杂志》 CSCD 北大核心 2010年第6期486-487,共2页 Chinese Journal of Practical Pediatrics
关键词 纯红细胞再生障碍性贫血 高免疫球蛋白M 综合征 原发性免疫缺陷病 中性粒细胞减少症 幼年特发性关节炎 自身免疫性疾病 中国医学科学院
分类号 R72 [医药卫生—儿科]
  • 相关文献

参考文献10

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二级参考文献1

  • 1Notarangelo L,Casanova JL,Conley ME,et al.Primary immunodeficiency diseases an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee Meeting in Budapest,2005[J].J Allergy Clin Immunol,2006,117(4):883-896.

共引文献3

同被引文献10

  • 1Notarangelo L D, Duse M, Ugazio A G. Immunodeficiency with hyper-IgM (HIM)[J]. Immunodefic Rev. 1992,3(2):101-121. 0.
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  • 5Malamut G, Ziol M, Suarez F, et al. Nodular regenerative hyperplasi- a : the main liver disease in patients with primary hypogammaglobuline- mia and hepatic abnormalities[ J]. J Hepatol, 2008,48 ( 1 ) : 74 - 82.
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  • 7Malhotra RK, Li W. Poorly differentiated gastroenteropancreatic neuro- endocrine carcinoma associated with X-linked hyperimmunoglobulin M syndrome[J]. Arch Pathol Lab Med, 2008, 132(5) : 847 -85.
  • 8Bhushan A, Barnhart B, Shone S, eta. A transcriptional defect underlies B lymphocyte dysftmction in a patient diagnosed with non-X-linked hyper- IgM syndrome[J]. J Immunol, 2000, 164(6) : 2871 -2880.
  • 9Fontana S, Moratto D, Mangal S, et al. Functional defects of dendritic cells in patients with CD40 deficiency [J]. Blood, 2003, 102 ( 12 ) :4099-4106.
  • 10Tavil B, Sanal O, Turul T, et al. Parvovirus B19-induced persistent pure red cell aplasia in a child with T-cell immunodeficiency[ J ]. Pe- diatr Hematol Oncol, 2009, 26 (2) : 63 -68.

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中国实用儿科杂志
2010年 第6期

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