Abstract
Within T-cell lymphomas (TCL) there are 2 entities expressing gamma-delta TCR: hepatosplenic gamma-delta T-cell lymphoma (HSGDTL) and the primary cutaneous gamma-delta T-cell lymphoma (PCGDTL). PCGDTL is a rare form of Tcell lymphoma with specific tropism for skin that have a dismal prognosis. Although even rarer, there have been reports of TCL with loss of expression of the TCR, which have been termed peripheral TCL TCR-silent type. We report the case of a cutaneous TCR-silent type lymphoma associated to a clonal plasma cell proliferation with an ominous outcome that led to a lot of discussion in its classification. Due to the aggressiveness of the disease and the scant evidence about therapy in this strange entity the outcome was fatal. We report a unique case of a TCR-silent cutaneous TCL with an exceptional histopathology, prolonged clinical evolution and a subsequent plasma cell clonal expansion.
Highlights
E even rarer, there have been s reports of T-cell lymphomas (TCL) with loss of expression of the T-cell receptor (TCR), which have been termed peripheru al TCL TCR-silent type
We report the case l of a cutaneous TCR-silent type lymphoma ia associated to a clonal plasma cell proliferation with an ominous outcome that led to a rc lot of discussion in its classification
We describe an infrequent form of disseminated cutaneous T-cell lymphomas (CTCLs) with loss of expression of the TCR (TCR silent type) associated with a clonal plasma cell expansion
Summary
E even rarer, there have been s reports of TCL with loss of expression of the TCR, which have been termed peripheru al TCL TCR-silent type. We report the case l of a cutaneous TCR-silent type lymphoma ia associated to a clonal plasma cell proliferation with an ominous outcome that led to a rc lot of discussion in its classification. We report a unique case of a TCR-silent cutaneous TCL
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