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双表型双基因型淋巴瘤,同时表达PAX5/BSAP以及B细胞和T细胞标志物。

Biphenotypic bigenotypic lymphoma with simultaneous expression of PAX5/BSAP and B- and T-cell markers.

作者信息

Hansson Markus, Jerkeman Mats, Dictor Michael

机构信息

Department of Hematology, Lund University Hospital, Lund, Sweden.

出版信息

Eur J Haematol. 2007 Aug;79(2):159-65. doi: 10.1111/j.1600-0609.2007.00882.x.

Abstract

Lymphomas are currently categorized according to their origin from a B or T lymphocyte. Immature and less commonly mature (peripheral) lymphomas may harbor rearrangements of both the B- and T-cell antigen receptor genes (dual genotype or bigenotype). Rarely, cells in lymphoma with a single genotype simultaneously express both B- and T-cell markers (biphenotypic lymphomas). We discuss the diagnostic and clinical implications in the case of a 42-yr-old female with a peripheral CD30+ lymphoma that displayed both characteristic B- and T-cell surface antigens and clonal rearrangement of B- and T-cell antigen receptor gene loci. Simultaneous nuclear expression of the transcription factor gene PAX5 suggested that this major driver of B-cell differentiation did not preclude expression of CD3epsilon, generally assumed to be a T-cell associated antigen.

摘要

淋巴瘤目前根据其起源于B淋巴细胞还是T淋巴细胞进行分类。不成熟以及较少见的成熟(外周)淋巴瘤可能同时存在B细胞和T细胞抗原受体基因重排(双基因型)。罕见的情况下,具有单一基因型的淋巴瘤细胞可同时表达B细胞和T细胞标志物(双表型淋巴瘤)。我们讨论了一名42岁女性外周CD30+淋巴瘤病例的诊断及临床意义,该淋巴瘤同时显示出特征性的B细胞和T细胞表面抗原以及B细胞和T细胞抗原受体基因位点的克隆重排。转录因子基因PAX5的同时核表达提示,这种B细胞分化的主要驱动因子并不排除通常被认为是T细胞相关抗原的CD3ε的表达。

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