Abnormal immunophenotype of the T-cell-receptor beta chain in follicular-helper T cells of angioimmunoblastic T-cell lymphoma

Objective Analysis for 24 variable regions of the T-cell-receptor beta chain by flow cytometry (Vbeta) is a new technique to detect clonal alpha-beta T lymphocytes and is characteristically performed on peripheral blood. Angioimmunoblastic T-cell lymphoma (AITL) has increased neoplastic follicular-helper T cells (FHT), which often express CD10; but nonneoplastic, CD10-positive T cells may be associated with reactive lymphadenopathy and with B-cell lymphomas. This study documents the utility of Vbeta analysis of FHT in specimens of AITL from blood, from marrow, and from lymph nodes. Methods The electronic medical record in the flow cytometry laboratory was searched for specimens that were analyzed by flow cytometry for Vbeta and that were involved by AITL. Flow cytometry was performed for the following antigens: T-cell-associated proteins, CD10, CD56, CD94, CD161, killer-cell immunoglobulin-like receptors, alpha-beta T-cell receptor, gamma-delta T-cell receptor, and Vbeta. Results Five patients had six specimens of blood (two), of bone marrow (one), or of lymph nodes (three). Immunophenotypic aberrances were detected for antigens: CD2 (2/6), CD3 (6/6), CD4 (1/6), CD5 (1/6), CD7 (5/6), and CD45 (2/6). All abnormal T-cell populations expressed CD4, and most expressed CD10 (5/6). Four specimens were clonally restricted for Vbeta. Two specimens lacked the alpha-beta T-cell receptor and Vbeta. Conclusions Vbeta analysis by flow cytometry can be used to detect clonal alpha-beta FHT in AITL, which may be difficult to diagnose with early involvement. Abnormal Vbeta expression on CD10-positive T cells confirms that FHT are the neoplastic cells.