Sézary syndrome with hyposplenism

Phenotypic, functional and clinical analysis of two patients with Sezary Syndrome are presented. Both patients had an elevated lymphocyte count with the helper/inducer cell phenotype by analysis with monoclonal antibodies (OKT3+, T4+, T6−, T8−, M1−), had the characteristic cerebriform nucleus of Sezary cells, and were Fc-IgG receptor negative. The functional tests revealed no proliferative, cytotoxic or immunoregulatory activity of patient E.P.'s leukemic cells, while the lymphocytes of patient A.N. responded to mitogen stimulation and had helper cell capacity in pokeweed mitogen driven B cell differentiation and maturation. Both patients presented skin involvement, pruritus, hepatomegaly and patient E.P. showed generalized lymphadenopathy. The spleen size of patient A.N. was below the normal range with an estimated spleen weight of ∼ 160g (normal 180–229g). Patient E.P. had an extremely small spleen size with an estimated weight of ∼ 20g as shown by abdominal sonography and spleen scintigraphy and had Howell-Jolly bodies within the erythrocytes. The size of the spleen in various other diseases with T cell proliferations is discussed with respect to the possible proliferative centers of the various T-cell subpopulations.