Primary cutaneous CD8+ epidermotropic cytotoxic T-cell lymphomas, a rare and dramatic challenge still open

Primary cutaneous CD8+ epidermotropic cytotoxic T-cell lymphomas, a rare and dramatic challenge still open
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Author: Andrea Duminuco; Laura Anastasia Caruso; Angelo Curto Pelle; Annalisa Chiarenza
Category: Lymphoma: Mature T and NK cell lymphoproliferations > Cutaneous T-cell Lymphoma > Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma
Published Date: 11/05/2021

We present a case of a 68-year-old patient, suffering from ulcerated/necrotic lesion initially present on the right leg (figure A), subjected to biopsy: infiltrate of atypical medium-sized lymphocytes is found in the dermal-epidermal interface, in the dermis and in the hypodermis, with angio- and neurotropism, widespread aspects of epidermotropism, lymphocytic exocytosis and numerous intraepithelial nests of atypical lymphocytes (Darier's nests). High growth fraction (Ki-67/Mib-1 approximately 60%) and immunophenotype CD3+, CD2+, CD7+, CD8+, Bcl-2+, CD43±, CD4-, CD5-, CD30-, CD56-, CD57- , CD10-, PD1-, CXCL13-, Bcl-6-, CD20- and CD19-, without clonal rearrangement of TCR chains. Diagnosis of primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma is made. This entity of 2016 WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues is a very rarely cutaneous T-cell lymphoma, characterized aggressive clinical behavior. The patient was initiated into therapy with different lines of treatment (e.g., local radiotherapy, bexarotene, extracorporeal photopheresis and gemcitabine). At the end of the 9th administration of gemcitabine, the skin appearance of the patient's limb had dramatically worsened (necrotic erosions with the presence of purulent and foul-smelling fluid, figure B), with bone marrow and inguinal lymph node involvement. Given significant disease’s progression, the patient was treated according to the CHOEP scheme, with improvement of the skin lesions and achievement of a complete response to treatment (figure C), thus ensuring the possibility of continuing his therapeutic process with ASCT. It is interesting to consider how this lymphoma is a very rare entity and possibly of difficult differential diagnosis, characterized by cutaneous manifestations with a clinical course rapidly and dramatically progressive and incapacitating for the patient. A prompt diagnosis and a correct therapeutic strategy are necessary to guarantee the patient the best prognosis and quality of life, considering that this lymphoma is characterized by a median survival of 12 months.