Angiocentric T - cell Lymphoma of the Skin / 대한피부과학회지

ABSTRACT

BACKGROUND: Angiocentric T-cell lymphomas are rare T-cell malignancies which involve extranodal sites, such as the skin, nasal cavity, soft tissue and gastrointestinal tract. They have been reported with significant frequency in Asia. OBJECTIVES: The main objective of this study is to characterize the clinical, pathological, and immunohistochemical featnres of cutaneous angiocentric T-cell lymphoma. Another objective is to search for the Epstein-Barr virus (EBV) in the tissues of cutaneous angiocentiric T cell lymphoma. METHODS: Clinical records, laboratory data, and histopathologic sections of 12 patients with cutaneous angiocentric T-cell lymphoma were reviewed. Paraffin tumor tissues were immunophenotyped. In situ hybridizaion studies were performed to detect the EBV genomes. RESULTS: The ages of the 12 patients ranged from 34 to 64 years(mean 45.8 years). The cutaneous lesions were nodules or plaqes, and were with ulcerated or had intact skin. Eight patients had evidence of extracutaneous involvement, usually involving lymph nodes, liver, and spleen. Eleven patients showed the abnormal laboratory findings including anemia, leukopenia, and elevated level of LDH. The disease pursued an aggressive course and was not uncommonly resistant to treatment. Histologically, the lymphomatous infiltrate occurred predominantly in the subcutaneous layer with involvement of the dermis. The pattern was mainly perivascular and periadnexal. A prominent feature was invasion of small or medium vesselsby lymphoma cells. The infiltrating lymphrcytes expressed CD45RO in all cases; variable expression of CD3 and CD56 was detected in piaffin sections. Among the 11 cases where in situ hybridization was performed, EBV genome could be detected in 9 cases. CONCLUSION: Angiocentric T-cell lymphoma of the skin is an aggressive lymphoma distinct from classic cutaneous T-cell lymphoma. However, further studies are needed to regard them as a homogeneous entity of T-cell lymphoma involving the skin.