Primary cutaneous gamma/delta-positive T-cell lymphoma

Primary cutaneous gamma/delta-positive T-cell lymphoma (PCGD-TCL) is a rare and aggressive type of non-Hodgkin lymphoma characterized by the proliferation of mature, activated gamma/delta T-cells that primarily involve the skin. This lymphoma was recognized as a distinct provisional entity by the World Health Organization (WHO) classification of lymphoid neoplasms. It predominantly affects the skin but can also disseminate to mucosal and other extranodal sites; lymph node and bone marrow involvement is uncommon. The disease typically presents with disseminated plaques, nodules, and ulceronecrotic skin lesions, often involving the extremities. Subcutaneous involvement resembling subcutaneous panniculitis-like T-cell lymphoma may also occur. Patients may experience systemic symptoms such as fever, weight loss, and fatigue. A hemophagocytic syndrome, a potentially life-threatening complication involving excessive immune activation, may develop during the disease course. The neoplastic cells express the gamma/delta T-cell receptor and typically have a cytotoxic phenotype, often expressing cytotoxic markers such as granzyme B and TIA-1. They are usually negative for CD4 and CD5, and variably express CD8. The epidermal, dermal, and subcutaneous tissue layers of the skin can all be involved. The prognosis of PCGD-TCL is generally poor, with a median survival reported at approximately 15 months, though outcomes vary depending on the extent of disease and specific clinical features. Treatment remains challenging, and there is no established standard of care. Therapeutic approaches include multi-agent chemotherapy regimens (such as CHOP-based protocols), radiation therapy for localized disease, and allogeneic hematopoietic stem cell transplantation in eligible patients. Novel agents and immunotherapies are being explored in clinical trials. Due to its rarity and aggressive nature, management is best guided by multidisciplinary teams at specialized centers.

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