title: Phenotypic evolution in a case of peripheral T-cell lymphoma suggests the presence of tumor heterogeneity.
abstract: Peripheral T-cell lymphoma (PTCL), not otherwise specified (NOS), represents a heterogeneous group of nodal and extranodal lymphomas that express a variety of T-cell antigens indicative of mature T-cell lineage. Most cases of PTCL express CD4 and lack CD8 expression and have a T-helper immunophenotype. Although the immunophenotype of PTCL is usually stable over time, immunophenotypic switch or evolution from T-helper to T-suppressor or vice versa has been rarely reported. Herein, we report a patient who presented with nasal PTCL, NOS, that was CD8+ and negative for Epstein-Barr virus, with concurrent skin lesions that had a CD8+/TIA-1+ T-cell immunophenotype. Patient received multi-agent chemotherapy and matched unrelated donor stem cell transplant, and subsequently suffered a cutaneous relapse with a CD4+/TIA-1(-) immunophenotype. Molecular analysis of the neoplasm biopsied at presentation showed one monoclonal T-cell receptor gamma gene rearrangement, and a second oligoclonal peak. At the time of CD4-positive recurrence, the oligoclonal peak was rather prominent, suggesting that the emergence of this peak is related with the phenotypic evolution from CD8+ to CD4+ predominant. These results highlight the utility of sequential immunophenotypic and molecular analysis of PTCL cases at the time of relapse to better understand the mechanisms of disease.
authors.affiliation: Department of Pathology and Laboratory Medicine, The University of Texas Medical School at Houston, Houston, TX, USA.
authors.forename: Alyaa
authors.initials: A
authors.lastname: Al-Ibraheemi
authors.forename: Rashmi
authors.initials: R
authors.lastname: Kanagal-Shamanna
authors.forename: Keyur P
authors.initials: KP
authors.lastname: Patel
authors.forename: Sergej N
authors.initials: SN
authors.lastname: Konoplev
authors.forename: Pramod
authors.initials: P
authors.lastname: Mehta
authors.forename: L Jeffrey
authors.initials: LJ
authors.lastname: Medeiros
authors.forename: Roberto N
authors.initials: RN
authors.lastname: Miranda
mesh_terms: D000328:Adult; D015496:CD4-Positive T-Lymphocytes / Q000378:metabolism / Q000473:pathology; D018414:CD8-Positive T-Lymphocytes / Q000378:metabolism / Q000473:pathology; D005260:Female; D006801:Humans; D016411:Lymphoma, T-Cell, Peripheral / Q000378:metabolism / Q000473:pathology; D009363:Neoplasm Proteins / Q000378:metabolism*; D016609:Neoplasms, Second Primary / Q000378:metabolism / Q000473:pathology; D009669:Nose Neoplasms / Q000378:metabolism / Q000473:pathology; D039102:Poly(A)-Binding Proteins / Q000378:metabolism*; D012878:Skin Neoplasms / Q000378:metabolism / Q000473:pathology; D000074019:T-Cell Intracellular Antigen-1
chemical_list: D009363:Neoplasm Proteins; D039102:Poly(A)-Binding Proteins; D000074019:T-Cell Intracellular Antigen-1; C071558:TIA1 protein, human