Durable remission of a patient with primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma

Abstract

Primary cutaneous CD8⁺ aggressive epidermotropic cytotoxic T-cell lymphoma (CD8⁺ PCAETL) is a rare disease characterized by aggressive clinical course and short survival. All available data are extracted from case reports and case series. The outcome is dismal and only two reported cases were cured after several lines of therapies including stem cell transplant. We herein present the case of a patient with CD8⁺ PCAETL who presented with rapidly progressive skin lesions and systemic symptoms. He was treated with aggressive multiagent chemotherapy comprising cyclophosphamide, vincristine, doxorubicin, and dexamethasone alternating with high-dose methotrexate and cytarabine (hyper-CVAD). The treatment resulted in durable complete remission with no evidence of disease recurrence after 58 months of follow-up. This is the first reported case of durable remission after first-line treatment.

Keywords: CD8(+) aggressive epidermotropic cytotoxic T-cell lymphoma; Hyper-CVAD; Lymphoma; Multiagent chemotherapy; World Health Organization.