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Case Reports

Abstract

Primary cutaneous peripheral T-cell lymphoma, not otherwise specified (pcPTCL-NOS) is a recently defined, poorly characterized entity representing a group of rare cases that do not meet criteria for any of the defined cutaneous T-cell lymphoma subtypes. We report a case of a 79-year-old male who was initially found to have a small erythematous plaque on the left earlobe. Shave biopsy showed an atypical CD3+ lymphoid infiltrate in the superficial dermis, with increased CD4:CD8 ratio and partial loss of CD7. T-cell clonality studies were notable for a clonal T-cell receptor gamma gene rearrangement. He was prescribed topical steroids but was briefly lost to follow-up, until he re-presented 2 years later with progressively worsening cervical, axillary, and inguinal lymphadenopathy. Core needle biopsy of a cervical lymph node was obtained, which revealed an infiltration of small to medium sized lymphoid cells. The neoplastic cells were positive for CD3, CD43, and CD5 and co-expressed CD4, PD-1, and GATA3. A clonal T-cell receptor gamma gene rearrangement was identified, with PCR products of the same size as those seen from the patient’s previous earlobe biopsy. Taken together, this was most consistent with a diagnosis of pcPTCL-NOS. The patient was treated with mini-CHOP chemotherapy with initial complete response, however he subsequently developed new diffuse lymph node enlargement found to be consistent with recurrent lymphoma. This case highlights the importance of close clinical follow-up in patients with rare types of primary cutaneous lymphomas, given the difficulty in predicting potential for disease progression based on histopathologic features alone.