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A patient with long standing seropositive rheumatoid arthritis developed lymphocytosis which phenotypically involved the cytotoxic/suppressor T-lymphocyte population. There are 10 reported instances of this new disease entity described as "chronic T-cell lymphocytosis with neutropenia" or "chronic suppressor T-cell lymphocytic leukemia." The disease is characterized by hepatosplenomegaly, neutropenia, and the frequent presence of rheumatoid factor without clinical evidence of rheumatoid arthritis. Splenectomy in our patient, as well as in other instances where undertaken, has been ineffective in alleviating the neutropenia. The peripheral blood lymphocytes in our patient were OKT-3+, OKT-5+, OKT-8+, OKT-11+, cALL-, OKT-6-, TdT-. They possessed ADCC but no NK activity and did not suppress PWM-induced B-cell differentiation in spite of the presence of Fc receptor for IgG. Since the lymphocytosis of OKT-8+ cells appears to be clonal, it is suggested that the disease be designated chronic suppressor T-cell lymphocytic leukemia.
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