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Case Report

Silent T-Cell Receptor Cutaneous T-Cell Lymphoma Associated to a Clonal Plasma Cell Proliferation

1
Department of Hematology, Hospital de la Santa Creu i Sant Pau, Carrer Mas Casanovas, 90 4th Floor, 08041 Barcelona, Spain
2
Pathology Department, Hospital de la Santa Creu i Sant Pau, 08041 Barcelona, Spain
3
Dermatology Department, Hospital de la Santa Creu i Sant Pau, 08041 Barcelona, Spain
*
Author to whom correspondence should be addressed.
Hematol. Rep. 2019, 11(1), 7841; https://doi.org/10.4081/hr.2019.7841
Submission received: 21 August 2018 / Revised: 1 December 2018 / Accepted: 7 December 2018 / Published: 19 February 2019

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 T-cell 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.
Keywords: silent TCR; gamma-delta T-cell lymphoma; PTCL silent TCR; gamma-delta T-cell lymphoma; PTCL

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MDPI and ACS Style

Caballero, A.; Novelli, S.; Mozos, A.; Garcia Muret, P.; Sierra, J.; Briones, J. Silent T-Cell Receptor Cutaneous T-Cell Lymphoma Associated to a Clonal Plasma Cell Proliferation. Hematol. Rep. 2019, 11, 7841. https://doi.org/10.4081/hr.2019.7841

AMA Style

Caballero A, Novelli S, Mozos A, Garcia Muret P, Sierra J, Briones J. Silent T-Cell Receptor Cutaneous T-Cell Lymphoma Associated to a Clonal Plasma Cell Proliferation. Hematology Reports. 2019; 11(1):7841. https://doi.org/10.4081/hr.2019.7841

Chicago/Turabian Style

Caballero, Ana, Silvana Novelli, Anna Mozos, Pilar Garcia Muret, Jorge Sierra, and Javier Briones. 2019. "Silent T-Cell Receptor Cutaneous T-Cell Lymphoma Associated to a Clonal Plasma Cell Proliferation" Hematology Reports 11, no. 1: 7841. https://doi.org/10.4081/hr.2019.7841

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