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免疫缺陷相关恶性淋巴瘤。三例显示T细胞和B细胞系基因证据的病例。

Immunodeficiency-associated malignant lymphoma. Three cases showing genotypic evidence of both T- and B-cell lineages.

作者信息

Hollingsworth H C, Stetler-Stevenson M, Gagneten D, Kingma D W, Raffeld M, Jaffe E S

机构信息

Laboratory of Pathology, National Cancer Institute, Bethesda, MD 20892.

出版信息

Am J Surg Pathol. 1994 Nov;18(11):1092-101.

PMID:7943530
Abstract

Dual rearrangement of both immunoglobulin and T-cell receptor (TCR) genes has been described in up to 30% of precursor lymphoid neoplasms, but this phenomenon occurs rarely in lymphomas with a mature phenotype. We describe three cases of immunodeficiency-associated lymphomas, two in patients with acquired immunodeficiency syndrome (AIDS) and one in a patient with a renal transplant, in which both immunoglobulin and TCR gene rearrangements were identified. Two cases exhibited clonal JH, clonal JK, and clonal TCR beta gene rearrangements; the other case had clonal JK and TCR beta gene rearrangements with deletion of JH. Two of these bigenotypic lesions exhibited a B-cell phenotype, and the other exhibited evidence of both a B- and T-cell phenotype, containing cytoplasmic CD3 as well as monoclonal kappa light chain. In addition, two of the cases exhibited the presence of clonal Epstein-Barr viral DNA. These observations suggest that lymphomas arising in immunodeficiency states may be more prone to disordered differentiation than those arising in an intact immune system.

摘要

高达30%的前体淋巴细胞肿瘤中已发现免疫球蛋白和T细胞受体(TCR)基因的双重重排,但这种现象在具有成熟表型的淋巴瘤中很少发生。我们描述了3例免疫缺陷相关淋巴瘤,2例发生在获得性免疫缺陷综合征(AIDS)患者中,1例发生在肾移植患者中,这些病例均检测到免疫球蛋白和TCR基因重排。2例表现为克隆性JH、克隆性JK和克隆性TCRβ基因重排;另一例有克隆性JK和TCRβ基因重排,JH缺失。这些双基因型病变中的2例表现为B细胞表型,另一例表现出B细胞和T细胞表型的证据,含有细胞质CD3以及单克隆κ轻链。此外,其中2例检测到克隆性EB病毒DNA。这些观察结果表明,免疫缺陷状态下发生的淋巴瘤可能比完整免疫系统中发生的淋巴瘤更容易出现分化紊乱。

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