title: Primary Cutaneous T-cell Lymphoma With Coexpression of T-Cell Receptors αβ and γδ.
abstract: T lymphocytes belong to 2 distinct sublineages that express either αβ or γδ T-cell receptor (TCR) complex. Although malignancy is a great instigator of lineage infidelity, as exemplified by aberrant expression of numerous lineage markers in lymphoma cells, malignant T cells rarely coexpress αβ and γδ TCR complexes. Similarly, only rare cases of CD4/CD8 double-positive primary cutaneous T-cell lymphoma have been reported. In this report, we describe a remarkable case of primary cutaneous T-cell lymphoma coexpressing αβ and γδ TCR complexes, strong diffuse CD8, and a very restricted coexpression of CD4 and CD8. A 66-year-old man was referred to our center for treatment of a persistent eczematoid eruption of 6 years of duration. An initial biopsy demonstrated not only marked spongiosis, but also an epidermotropic population of CD4 small mature T cells with partial expression of CD8. The process remained indolent for another year, followed by an abrupt progression with development of plaques and tumors. Repeat biopsies of these lesions demonstrated a superimposed population of large anaplastic T cells extensively involving the dermis and epidermis. The large cells showed a strong uniform expression of CD3, CD8, CD45RA, CD5, granzyme, TIA1, perforin, TCR-β, and TCR-γ and a weaker but unambiguous expression of CD4, CD25, CD2, and CD56. TCR gene rearrangement studies showed clonal rearrangements for TCR-β and TCR-γ with identical peaks to those seen in the biopsy from a year earlier. The patient developed lymphadenopathy, with a biopsy showing nodal involvement by a morphologically and phenotypically identical neoplastic T-cell population. The disease showed partial response to systemic chemotherapy with development of new plaques, but these new lesions have regressed with radiation therapy.
authors.affiliation: Departments of *Pathology, †Urology, and ‡Dermatology, University of Alabama at Birmingham, Birmingham, AL.
authors.forename: Vishwas
authors.initials: V
authors.lastname: Parekh
authors.forename: Eun-Hee
authors.initials: EH
authors.lastname: Shim
authors.forename: Charles F
authors.initials: CF
authors.lastname: Knapp
authors.forename: Lauren
authors.initials: L
authors.lastname: Hughey
authors.forename: Craig A
authors.initials: CA
authors.lastname: Elmets
authors.forename: Kristopher
authors.initials: K
authors.lastname: McKay
mesh_terms: D000368:Aged; D015703:Antigens, CD / Q000032:analysis*; D053804:Granzymes / Q000032:analysis; D006801:Humans; D016410:Lymphoma, T-Cell, Cutaneous / Q000737:chemistry* / Q000473:pathology*; D008297:Male; D054353:Perforin / Q000032:analysis; D039102:Poly(A)-Binding Proteins / Q000032:analysis; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000032:analysis*; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000032:analysis*; D000074019:T-Cell Intracellular Antigen-1
chemical_list: D015703:Antigens, CD; D039102:Poly(A)-Binding Proteins; D016693:Receptors, Antigen, T-Cell, alpha-beta; D016692:Receptors, Antigen, T-Cell, gamma-delta; D000074019:T-Cell Intracellular Antigen-1; C071558:TIA1 protein, human; D054353:Perforin; D053804:Granzymes