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Peripheral T-Cell Lymphoma with Aberrant Expression of CD79a and CD20: A Diagnostic Pitfall

机译:CD79a和CD20异常表达的外周T细胞淋巴瘤:诊断误区

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Immunohistochemical studies are increasingly used for the routine diagnosis of lymphomas as it is widely accepted that lymphomas of different cell lineages vary in their prognosis and response to therapy. A case of peripheral T-cell lymphoma with aberrant expression of B-cell–associated antigens L-26 (CD20) and mb-1 (CD 79a) is described. The disease pursued an aggressive clinical course, and the patient died of disease 6 weeks after presentation.Immunohistochemical studies demonstrated expression of both T- and B-cell–associated antigens, including CD3, CD8, CD43, TIA-1, CD20, and CD79a. Other markers expressed by the tumor cells included CD56 and S-100. Of interest, F-1 staining for the chain of T-cell receptor (TCR) complex was positive in the small admixed T lymphocytes but was negative in the tumor cells, raising the possibility of a / T-cell lymphoma. Molecular studies by polymerase chain reaction (PCR) demonstrated clonal TCR- chain gene rearrangement without evidence for a clonal rearrangement of the immunoglobulin heavy chain gene. PCR for HHV-8 related sequences was negative.Mb-1 is an IgM-associated protein that was thought to be restricted to normal and neoplastic B cells. Although its coexpression has been reported in up to 10% cases of precursor T-cell lymphoblastic lymphoma, the coexpression of both CD20 and CD79a has not been described in mature T-cell malignancies. Biphenotypic lymphomas associated with HHV-8 have been reported in immunodeficiency, but no evidence of immune deficiency was identified, and studies for EBV and HHV-8 were negative. This case illustrates that no marker has absolute lineage specificity and that immunophenotypic studies should always be performed with panels of monoclonal antibodies. Moreover, cases with ambiguous phenotypes may require genotypic studies for precise lineage assignment.
机译:免疫组织化学研究越来越多地用于淋巴瘤的常规诊断,因为人们普遍认为不同细胞谱系的淋巴瘤的预后和对治疗的反应会有所不同。描述了一例伴有B细胞相关抗原L-26(CD20)和mb-1(CD 79a)异常表达的外周T细胞淋巴瘤。该疾病遵循积极的临床过程,患者在就诊后6周就死于疾病。免疫组织化学研究表明T细胞和B细胞相关抗原均表达,包括CD3,CD8,CD43,TIA-1,CD20和CD79a 。肿瘤细胞表达的其他标志物包括CD56和S-100。有趣的是,T细胞受体(TCR)复合物链的F-1染色在小的混合T淋巴细胞中呈阳性,而在肿瘤细胞中呈阴性,从而增加了T细胞淋巴瘤的可能性。通过聚合酶链反应(PCR)进行的分子研究表明,克隆的TCR-链基因重排没有证据表明免疫球蛋白重链基因的克隆重排。 HHV-8相关序列的PCR阴性.Mb-1是IgM相关蛋白,被认为仅限于正常B细胞​​和肿瘤B细胞。尽管在多达10%的前体T细胞淋巴母细胞淋巴瘤病例中已经报道了其共表达,但在成熟的T细胞恶性肿瘤中尚未描述CD20和CD79a的共表达。已经报道了与HHV-8相关的双表型淋巴瘤存在免疫缺陷,但没有发现免疫缺陷的证据,EBV和HHV-8的研究均为阴性。这种情况说明没有标记具有绝对的血统特异性,免疫表型研究应始终与单克隆抗体组一起进行。此外,具有歧义表型的病例可能需要进行基因型研究以准确确定血统。

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