Hematopoietic stem cell transplantation in primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma: A case report

Abstract

Primary Cutaneous Aggressive Epidermotropic CD8+ T-Cell Lymphoma (PCAETCL), a rare subtype of Primary Cutaneous Lymphoma (PCL), is known for its aggressive clinical course and refractoriness to standard treatments. Clinically, PCAETCL often presents with skin lesions that rapidly disseminate to various extracutaneous sites, resulting in a grim prognosis with a 5-year survival rate of less than 40%. This article presents a case of a 43-year-old male patient initially diagnosed with CD8+ cytotoxic Mycosis Fungoides (MF). The patient’s treatment course involved multiple therapeutic modalities, including methotrexate, bexarotene, gemcitabine, peginterferon alfa 2a, and radiation therapy. Despite several lines of treatment, disease progression persisted. Eventually, the patient underwent allogeneic Hematopoietic Stem Cell Transplantation (allo-HSCT) from his sister, marking a turning point in his therapeutic approach. The case underscores the diagnostic and therapeutic challenges associated with PCAETCL. It shows the heterogeneous responses found in literature, ranging from brief remissions to unfavorable outcomes, including severe infections. This highlights the lack of standardized treatment protocols for PCAETCL, with HSCT, both allogeneic and autologous, emerging as potential options for achieving prolonged responses. In conclusion, PCAETCL remains a rare and aggressive clinical entity with no established standard treatment. This case report illustrates the aggressive nature of the disease and the importance of individualized treatment approaches, including transplantation. Collaborative research efforts are crucial to enhance our understanding of PCAETCL, identify prognostic factors, and develop more effective therapeutic strategies to improve patient outcomes. Keywords: Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma Lymphoma; T-Cell; Cutaneous; Allogeneic hematopoietic stem cell transplantation Mycosis Fungoides; Extracutaneous manifestation Central nervous system involvement Multiple lines of therapy.