Monoclonal gammopathy in Sezary syndrome. Report of three cases and review of the literature

Abstract

Three patients with Sézary syndrome had monoclonal gammopathy: two had gammopathy of unknown significance and one had myeloma. The frequency of the association of Sézary syndrome and monoclonal gammopathy seems to be lower than previously suggested. However, it does emphasize that the presence of T-cell cutaneous disease does not preclude B-cell clonal proliferation.

Leser‐Trélat Sign Associated with Sézary Syndrome

“…The IgE level remained high throughout the course. The occurrence of the other monoclonal gammopathies has also been reported in 55 (26,27). We suggest that the high levels of IgE and IgG in our patient were not induced by malignant changes of B lymphocytes or plasma cells, but by secondary malignant biclonal immunoglobulinemia due to malignant proliferation of clone specific helper T lymphocytes, as suggested in the previous literature.…”

Monoclonal Gammopathy of Unknown Significance and Malignant Paraproteinemia in Hong Kong

“…An association with viral infections such as HTLV-1 and Epstein-Barr virus has been identified in some T-cell leukemias and lymphomas, 23 " 25 but the higher incidence in Southeast Asia of most T-cell Iymphoproliferative disorders remains an intriguing enigma, as does their occasional association with a paraprotein. Although not a common finding, paraproteinemia has been described previously in association with T-cell lymphomas such as angioimmunoblastic lymphadenopathy (AILD), 26 mycosis fungoides, 27 and adult T-cell leukemia/lymphoma. 28 ' 29 An association with plasma cell dyscrasia and lymphoplasmacytoid proliferation, possibly due to altered immunoregulation, has also been noted.…”

Tumor Stage Mycosis Fungoides in a Patient Treated with Long‐Term Corticosteroids for Asthma and Atopic‐Like Dermatitis

“…Elevated IgE levels have also been observed in other disorders of chronic antigen stimulation as well as in MF 3,4 in which antigen persistence may play an etiologic role 5 . Several cases of monoclonal gammopathy and less commonly of multiple myeloma occurring in patients with the Sézary syndrome suggests that, in some cases, neoplastic T lymphocytes may promote B lymphocyte activity and proliferation 13 …”