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抗体缺陷中的淋巴增殖性疾病:一项多中心研究。

Lymphoproliferative disease in antibody deficiency: a multi-centre study.

作者信息

Gompels M M, Hodges E, Lock R J, Angus B, White H, Larkin A, Chapel H M, Spickett G P, Misbah S A, Smith J L

机构信息

Immunology and Immunogenetics, Southmead Hospital, North Bristol NHS Trust, Bristol, UK.

出版信息

Clin Exp Immunol. 2003 Nov;134(2):314-20. doi: 10.1046/j.1365-2249.2003.02253.x.

DOI:10.1046/j.1365-2249.2003.02253.x
PMID:14616793
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1808874/
Abstract

We have undertaken a retrospective study of antibody deficient patients, with and without lymphoma, and assessed the ability of specific polymerase chain reaction (PCR) primers to determine if the detection of clonal lymphocyte populations correlates with clinical and immunohistochemical diagnosis of lymphoma. We identified 158 cases with antibody deficiency presenting during the past 20 years. Paraffin-embedded biopsy specimens or slides were available for analysis in a cohort of 34 patients. Of these patients, 29 had common variable immunodeficiency, one X-linked agammaglobulinaemia, one X-linked immunoglobulin deficiency of uncertain cause and three isolated IgG subclass deficiency. We have confirmed that lymphoma in antibody deficiency is predominantly B cell in origin. Clonal lymphocyte populations were demonstrated in biopsies irrespective of histology (16/19 with lymphoma and 11/15 without). Isolated evidence of clonality in biopsy material is therefore an insufficient diagnostic criterion to determine malignancy. Furthermore, our data suggest that clonal expansions are rarely the result of Epstein-Barr virus-driven disease.

摘要

我们对患有和未患淋巴瘤的抗体缺陷患者进行了一项回顾性研究,并评估了特异性聚合酶链反应(PCR)引物确定克隆淋巴细胞群的检测是否与淋巴瘤的临床和免疫组化诊断相关的能力。我们确定了过去20年中出现抗体缺陷的158例病例。在一组34例患者中,有石蜡包埋的活检标本或切片可供分析。在这些患者中,29例患有常见可变免疫缺陷,1例X连锁无丙种球蛋白血症,1例病因不明的X连锁免疫球蛋白缺乏症,3例孤立性IgG亚类缺乏症。我们已经证实,抗体缺陷患者中的淋巴瘤主要起源于B细胞。无论组织学如何,活检中均显示出克隆淋巴细胞群(19例淋巴瘤患者中有16例,15例未患淋巴瘤患者中有11例)。因此,活检材料中孤立的克隆性证据不足以作为确定恶性肿瘤的诊断标准。此外,我们的数据表明,克隆性扩增很少是由爱泼斯坦-巴尔病毒驱动的疾病所致。

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