Primary Cutaneous Gamma-Delta T-Cell Lymphoma: Case Report of a Rare Lymphoma and a Brief Review of the Literature

Primary cutaneous gamma-delta T-cell lymphoma (PCGDTCL) is a rare and highly aggressive subtype of cutaneous T-cell lymphoma characterized by rapid progression, resistance to therapy, and poor prognosis. We report the case of a 66-year-old man with a four-month history of rapidly progressive, painful ulcerative cutaneous lesions involving the lower extremities and trunk. The patient also reported systemic “B” symptoms, including fever, weight loss, and drenching night sweats.

Physical examination revealed multiple violaceous plaques and necrotic nodules without clinically evident lymphadenopathy or hepatosplenomegaly. Laboratory evaluation demonstrated pancytopenia, elevated lactate dehydrogenase levels, and hypoalbuminemia. Positron emission tomography–computed tomography (PET/CT) showed intense fluorodeoxyglucose uptake limited to the skin and subcutaneous tissues.

Histopathologic examination revealed dense atypical lymphocytic infiltration of the dermis and subcutaneous tissue with areas of necrosis and angioinvasion, sparing the epidermis. Immunophenotyping demonstrated a cytotoxic T-cell phenotype (CD3+, CD2+, CD56+, CD4−, CD8−) with a high proliferative index. Molecular analysis confirmed clonal rearrangement of the T-cell receptor gamma gene, establishing the diagnosis of PCGDTCL.

Despite treatment with CHOEP (Cyclophosphamide, Doxorubicin, Hydroxydaunorubicin, Vincristine Oncovin, Etoposide, and Prednisone) chemotherapy followed by second-line therapy, the disease progressed rapidly. It was complicated by hemophagocytic lymphohistiocytosis, leading to death 11 months after diagnosis. This report highlights the aggressive clinical course, clinicopathologic features, and underlying pathophysiology of PCGDTCL, and compares this case with findings from the current literature.