Sezary's syndrome: a clinical evaluation of eight patients

Abstract

Clinical and laboratory investigations of 8 patients with Sézary's syndrome are presented. Sézary cells were found in peripheral blood as well as in bone marrow and lymph nodes. The overall follow-up period was 8 years. Different therapeutic regimens were given. The best results were obtained with chlorambucil and prednisone orally, combined with topical application of nitrogen mustard. Three patients died of pneumonia and septicaemia.

Monoclonal gammopathies in sézary syndrome: A report of four new cases and a review of the literature

“…Other authors have failed to find monoclonal immunoglobulins in a series of eight patients with SCzary syndrome . 19 The summary of all nine clearly documented cases with Skzary syndrome and monoclonal gammopathy (Table 1) 20 The high frequency of IgA and IgM peaks as well as the low incidence of B-cell malignancies may indicate a pathogenetic relationship between monoclonal gammopathy and SCzary syndrome. Further evidence is provided from our data, since monoclonal gammopathy was observed more frequently (4/13) than could be expected from its natural incidence (2% among 70-79 year-old patients).…”

Sézary syndrome: Immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC)

“…Continuous treatment with chlorambucil (2-6 mg/d) in conjunction with prednisone (initially ;20 mg/d, tapering over time) (regimen of Winkelmann et al 169,224 ) resulted in a significant response in patients with SS. 223 In the study reported by McEvoy et al, 225 leukapheresis was also performed concurrently with an OR of 100% including two of 11 CR. MRD was 1 to 3 years and mean time to death was 6.5 to 8 years versus historical controls of 3 years.…”

“…[220][221][222] More extensive trials have been conducted for SS using chlorambucil (Table I). 97,223,224 In all these studies, oral corticosteroid has also been used in conjunction with chlorambucil to maintain responses and duration of improvement. In these open trials, all patients had skin and blood involvement and some had clinical evidence of nodal disease as well.…”

Other Chemotherapeutic Agents in Cutaneous T-Cell Lymphoma

“…Chlorambucil was one of the first systemic treatments recognized to have activity specifically in SS 38,39 and since that time, there have been multiple case reports/series suggesting benefit in this setting. [40][41][42] Winkelmann developed the Winkelmann method, which consists of oral chlorambucil (2-6 mg/d) with prednisone (10-20 mg/d, weaned over time) for the treatment of SS. 39,[43][44][45] It has also been adapted for the treatment of CLL, where it continues to be a common therapy.…”