[Rapidly progressive T-cell angiocentric lymphoma with CD56+ phenotype involving bilateral orbits and skin in a patient with anti-HTLV-I antibody].

A 57-year-old woman was admitted to our hospital complaining of left exophthalmos in July, 1991. Biopsy specimen of a tumor in the left orbit suggested an inflammatory pseudotumor. The orbit was irradiated and the exophthalmos disappeared. During admission, an erythema was noted on her anterior chest wall. After discharge, right exophthalmos and generalized skin eruptions developed. The skin eruption on her anterior chest wall was histopathologically diagnosed as angiocentric lymphoma and she was readmitted. Since her serum was positive for anti-HTLV-I antibody, adult T-cell leukemia/lymphoma was suspected. Chemotherapy yielded limited relief of the right exophthalmos and transient fading of the skin eruptions. She died due to progression of the disease. Close examination of the skin eruption on the calf revealed that the lymphoma cells were CD56-positive immunohistochemically and electron microscopy revealed that they had large membrane-bound granules. Rearrangement of the T-cell receptor beta chain gene was observed. Incorporation of HTLV-I proviral DNA was not observed. Re-examination of the left orbital tumor confirmed the infiltration of these lymphoma cells. This is a rare case of T-cell lymphoma with NK cell phenotype involving orbits and skin, and showing a rapid clinical course.