1989 Volume 30 Issue 7 Pages 1067-1073
We treated 54-year-old Japanese man with a large cell type of Sézary syndorome. He had generalized erythrodermia, superficial lymphadenopathy, atypical lymphocytes in the peripheral blood, anti-HTLV-I antibody negativity and chromosomal abnormality. The patient was a hepatitis B virus carrier, and was complicated with hepatocellular carcinoma and monoclonal gammopathy of IgG, λ type. Sézary syndrome is a T cell malignancy, the clinical course of which is relatively mild and chronic; accordingly, this case showed no crisis under chemotherapy. However, the patient died due to rapid growth of the hepatoma. Although case reports of Sézary syndrome complicated with other malignancies are very few, the occurrence of malignancies is possible because of decreased immunological function in the patients. In this case, hepatitis B virus might participate in the hepatic oncogenesis under dysfunction of helper/inducer cells. In addition, the complication of monoclonal gammopathy was also interesting from the standpoint of the helper function of Sézary cells.
