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获得性高IgM综合征伴坏死性肉芽肿。

Acquired hyper-IgM syndrome with necrotizing granuloma.

作者信息

Goldstein M F, Kornstein M J, Talbot S, Levinson A I

出版信息

J Allergy Clin Immunol. 1985 Apr;75(4):472-8. doi: 10.1016/s0091-6749(85)80020-8.

DOI:10.1016/s0091-6749(85)80020-8
PMID:3980882
Abstract

This article is highlighted by the finding of striking cervical lymphadenopathy in a patient with acquired hyper-IgM syndrome and the pathologic description of the involved nodes. Routine hematoxylin-eosin stains demonstrated the presence of idiopathic necrotizing granulomas in the nodal tissue, a finding not previously reported in this syndrome. Immunoperoxidase techniques were used to further characterize these granulomas and delineate the cellular composition of the nodal architecture. We found that the necrotizing granulomas consisted of a peripheral rim of Ia positive palisaded, epithelioid histiocytes and central areas of debris and scattered inflammatory cells that were T11 positive. In the uninvolved areas of the node, we observed a lack of IgG-bearing lymphocytes in germinal centers as well as an absence of IgG-containing and decreased IgA-containing plasma cells in interfollicular areas. In conjunction with these in situ observations, there was a lack of IgA and IgG immunoglobulin-secreting cell responses in pokeweed mitogen-stimulated cultures of the patient's peripheral blood mononuclear cells. Unique features of this article include: (1) the association of necrotizing granulomas with the hyper-IgM syndrome and (2) the use of monoclonal antibodies to characterize the distributions of nodal lymphocytes in a patient with this disorder.

摘要

本文重点介绍了一名获得性高IgM综合征患者出现显著颈部淋巴结病的发现以及受累淋巴结的病理描述。常规苏木精-伊红染色显示淋巴结组织中存在特发性坏死性肉芽肿,这一发现此前在该综合征中未见报道。采用免疫过氧化物酶技术进一步表征这些肉芽肿并描绘淋巴结结构的细胞组成。我们发现坏死性肉芽肿由Ia阳性栅栏状上皮样组织细胞构成的外周边缘和中央的碎片区域以及散在的T11阳性炎性细胞组成。在淋巴结未受累区域,我们观察到生发中心缺乏携带IgG的淋巴细胞,以及滤泡间区域缺乏含IgG和含IgA浆细胞减少。结合这些原位观察结果,患者外周血单个核细胞经商陆有丝分裂原刺激培养后缺乏IgA和IgG免疫球蛋白分泌细胞反应。本文的独特之处包括:(1)坏死性肉芽肿与高IgM综合征的关联;(2)使用单克隆抗体表征该疾病患者淋巴结淋巴细胞的分布。

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引用本文的文献

1
Cervical lymph node diseases in children.儿童颈部淋巴结疾病
GMS Curr Top Otorhinolaryngol Head Neck Surg. 2014 Dec 1;13:Doc08. doi: 10.3205/cto000111. eCollection 2014.