A CUTANEOUS AGRANULAR CD2(-) CD4(- REPORT OF 2 CASES AND REVIEW OF THE LITERATURE() CD56(+) LYMPHOMA )

We report 2 cases of agranular CD2(-) CD4(+) CD56(+) non-Hodgkin lymph oma in which skin seemed to be the primary site. A 21-year-old woman's initial symptom was a skin nodule on the right cheek. She also had tu mors in the nasopharynx, and the bone marrow subsequently became invol ved. No lymphadenopathy was present. She experienced complete remissio n after dose-intensified therapy with cyclophosphamide, hydroxydaunomy cin, vincristine [Oncovin], and prednisone (CHOP), but the disease rel apsed in the central nervous system 6 months later An 81-year-old man experienced an 11-month history of sk cin nodules in the left forearm. On admission, he had a bone marrow infiltration of lymphoma cells. He died of pneumonia during chemotherapy. The malignant cells of the 2 p atients had similar morphologic features, with a monocytoid nucleus an d no cytoplasmic granules. The cells in both cases showed a unique phe notype: CD2(-), CD3(-), CD4(+), CD8(-), CD13(-); CD14(-), CD34(-), CD1 6(-), CD56(+), CD57(-), HLA-DR-positive. Staining for peroxidase and a lpha-naphthyl butyrate esterase was negative. The T-cell receptor beta , gamma, delta, IgH, kappa, lambda genes were of germ line configurati ons. The DNA of Epstein-Barr virus was not detected from the bone marr ow cells by polymerase chain reaction. Only 3 other cases with similar phenotypes have been reported; all had skin lesions. Although the ori gin of these cells remains unknown, we propose that this is a distinct clinicopathologic entity.