Peripheral T cell lymphoma, NOS with aberrant αβ and γδ T cell receptor expression in a post-heart transplant patient

Abstract

Three main patterns of T cell receptor (TCR) expression are identified in peripheral T cell lymphoma (PTCL): TCR-αβ type, TCR-γδ type, and TCR silent type. Cases of PTCL that express both αβ and γδ receptors are rare. Here, we report a case of PTCL-NOS (not otherwise specified) with dual αβ and γδ expression in a 61-year-old female 6 years after orthotopic cardiac transplant. The patient presented with fatigue, fever, worsening anemia, and thrombocytopenia. A chest CT showed multiple irregular lung nodules and diffuse lymphadenopathy. Cytology evaluation of the lymph nodes revealed large atypical lymphocytes with immunopositivity for CD3, CD8, CD56, TIA1, and granzyme B and immunonegativity for CD5 and CD30. Epstein-Barr virus (EBV) was not detected by EBV-encoded RNA (EBER) in situ hybridization study. The corresponding flow cytometry demonstrated abnormal T cells with TCR-αβ and TCR-γδ expression and loss of CD5 expression. A TCR gene rearrangement study demonstrated the presence of a clonal lymphoid population with TCRβ and TCRγ rearrangements. The patient was deceased within 2 months despite aggressive treatment. The findings were consistent with an EBV-negative, monomorphic T cell post-transplant lymphoproliferative disorder (PTLD) with features of PTCL-NOS. A literature review of T cell lymphoma with co-expression of TCR-αβ and TCR-γδ was performed. A case of aggressive PTCL with dual expression of TCR-αβ and TCR-γδ in a post-transplant setting has not been previously reported.