A Skin Plaque Preceding Systemic Relapse of Gamma-Delta Hepatosplenic T-Cell Lymphoma

Hepatosplenic T-cell lymphoma (HSTL) is an uncommon, aggressive peripheral T-cell lymphoma with a dismal prognosis, usually expressing gamma-delta T-cell receptor on immunohistochemical study. We report the second instance in the literature of a solitary skin nodule heralding recurrence of HSTL. The patient was a 40-year-old man in apparent remission from HSTL, 4 years after chemotherapy and autologous bone marrow transplant. Biopsy of a flank lesion showed atypical lymphoid cells involving the dermis with a perivascular and periadnexal pattern, and fat lobules of the subcutaneous tissue. Their phenotype mirrored that of previous biopsies, with expression of CD2, CD3, CD7, CD56, and T-cell receptor-gamma, and lack of T-cell receptor-beta, CD4, CD5, and CD8. Cutaneous involvement by HSTL has rarely been reported either at initial diagnosis or at recurrence, and represents a diagnostic pitfall for primary cutaneous gamma-delta T-cell lymphoma.