Primary cutaneous T-cell lymphomas (CTCL) are a heterogeneous group of T-cell lymphoproliferative diseases that involve mainly the skin and are characterized by features of their diagnosis, clinical course and therapeutic approach. They include mainly fungal mycosis and CD30 + lymphoproliferative skin diseases (primary cutaneous anaplastic large cell lymphoma and lymphomatoid papulosis) which account for >50% of CTCL and primary cutaneous peripheral T-cell lymphoma, unspecified/ /not otherwise specified (PTL NOS) which occurs extremely rare. Activation antigen CD30 is a cell membrane glycoprotein that belongs to tumor necrosis factor (TNF) superfamily. Tumor cells in primary skin CD30-positive skin lymphomas express CD30 in more than 75%; in other nosological units it also can be detected but to a lesser extent. Most patients with cutaneous CD30 + lymphoproliferative diseases have an indolent the disease course of the disease with a favorable prognosis. Refractory course occurs in approximately 30% patients, and in 8% of cases lymphoma results in deaths. Recently monoclonal antibodies have been included in clinical practice for the treatment of T-cell lymphomas, one of which is brentuximab vedotin, a CD30 monoclonal antibody conjugated to monomethyl auristatin E. This article provides the clinical case of a patient with a refractory form of PTL NOS.
Lethal midline granuloma (peripheral T-cell lymphoma) after lymphomatoid papulosis