A Case of Adult T-cell Leukemia/Lymphoma (ATLL) with Specific Features of Panniculitis-like Lesions

Abstract

A case of ATLL in an 81-year-old woman is reported. She noticed redness and swelling on the right thigh and diffuse erythema on the entire body around August 2000. She was hospitalized in December 2000 with such findings as redness and swelling on the right thigh with local heat and tenderness, pitting edema on the right leg, and inguinal regional lymph node swelling. Laboratory findings included positive reactivity to HTLV-1 antibody and higher titer of soluble IL-2 receptor. Histopathological examination revealed dense infiltration of numerous neoplastic lymphocytes with conspicuous atypia and mitosis from the dermis to the subcutaneous fatty tissue, suggesting a diagnosis of ATLL. Monoclonal integration of HTLV-1 proviral DNA and existence of defective types of HTLV-1 proviruses in the affected tissues were observed in a sample of a subcutaneous lesion by Southern blot technique. Those results confirmed the diagnosis of ATLL with panniculitis-like lesions. Cases of T cell lymphoma with panniculitis-like lesions are well known, but those of ATLL are rare. Review of the literature revealed only four such cases, including our case, in Japan. In two, EB-virus encoded small RNA (EBER) was found in the tumor cells. However, in the present case, EBER was negative by the in situ hybridization method using skin lesion and regional lymph-node tissues. We reported here a case of ATLL with panniculitis-like lesions in which EB viruses were not involved. The clinical symptoms were resistant to various therapies. Her course was poor with a short survival period of 14 months after hospitalization.