Yao X, Teruya-Feldstein J, Raffeld M, Sorbara L, Jaffe E S
Hematopathology Section, Laboratory of Pathology, National Cancer Institute, Bethesda, Maryland, USA.
Mod Pathol. 2001 Feb;14(2):105-10. doi: 10.1038/modpathol.3880265.
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, betaF-1 staining for the beta 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 gamma/delta T-cell lymphoma. Molecular studies by polymerase chain reaction (PCR) demonstrated clonal TCR-gamma 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.
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在预后和对治疗的反应方面存在差异。本文描述了一例外周T细胞淋巴瘤,其异常表达B细胞相关抗原L-26(CD20)和mb-1(CD79a)。该疾病临床病程呈侵袭性,患者在就诊后6周死于疾病。免疫组织化学研究显示T细胞和B细胞相关抗原均有表达,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β链的βF-1染色在小的混合T淋巴细胞中呈阳性,但在肿瘤细胞中呈阴性,提示可能为γ/δT细胞淋巴瘤。聚合酶链反应(PCR)分子研究显示克隆性TCR-γ链基因重排,而免疫球蛋白重链基因无克隆性重排证据。HHV-8相关序列的PCR检测为阴性。Mb-1是一种与IgM相关的蛋白,曾被认为仅限于正常和肿瘤性B细胞。虽然其共表达在高达10%的前体T细胞淋巴母细胞淋巴瘤病例中已有报道,但CD20和CD79a在成熟T细胞恶性肿瘤中的共表达尚未见描述。免疫缺陷患者中曾报道过与HHV-8相关的双表型淋巴瘤,但未发现免疫缺陷证据,EBV和HHV-8研究均为阴性。该病例说明没有标志物具有绝对的谱系特异性,免疫表型研究应始终使用单克隆抗体组合进行。此外,表型不明确的病例可能需要进行基因分型研究以精确确定谱系。