Primary cutaneous T-cell lymphoma

Primary cutaneous T-cell lymphoma (CTCL) is a heterogeneous group of non-Hodgkin lymphomas characterized by the clonal proliferation of malignant T-lymphocytes that primarily involve the skin. The most common subtypes include mycosis fungoides and Sézary syndrome, but the group also encompasses primary cutaneous CD30-positive lymphoproliferative disorders, subcutaneous panniculitis-like T-cell lymphoma, and other rarer variants. CTCL predominantly affects the skin but can progressively involve lymph nodes, blood, and internal organs in advanced stages. Early-stage disease typically presents as persistent patches, plaques, or erythematous lesions on the skin that may be mistaken for eczema or psoriasis. Patients often experience intense pruritus (itching), and skin lesions may evolve over months to years. In Sézary syndrome, patients develop generalized erythroderma (widespread reddening of the skin), lymphadenopathy, and circulating malignant T-cells (Sézary cells) in the peripheral blood. The exact cause of primary cutaneous T-cell lymphoma remains unclear, though it is generally considered a sporadic condition without a well-defined hereditary pattern. Environmental exposures, chronic antigenic stimulation, and certain viral infections have been investigated as potential contributing factors, but no definitive causative agent has been established. The disease predominantly affects adults, with a median age of onset typically in the fifth to sixth decade of life, and it is slightly more common in males. Treatment depends on the subtype and stage of disease. Early-stage mycosis fungoides is often managed with skin-directed therapies including topical corticosteroids, phototherapy (PUVA or narrowband UVB), topical nitrogen mustard (mechlorethamine), and topical retinoids. More advanced or refractory disease may require systemic therapies such as oral retinoids (bexarotene), histone deacetylase inhibitors (vorinostat, romidepsin), interferons, monoclonal antibodies (mogamulizumab, brentuximab vedotin), extracorporeal photopheresis, or conventional chemotherapy. Allogeneic stem cell transplantation may be considered in select younger patients with aggressive or refractory disease. While early-stage CTCL often follows an indolent course with a favorable prognosis, advanced-stage disease carries a significantly reduced life expectancy.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Primary cutaneous T-cell lymphoma