The treatment of Sézary syndrome

The results of treatment programs were studied in forty patients with Sézary syndrome. Thirty-three patients died. The principal treatment was a low-dose chlorambucil and prednisone regimen. Patients so treated lived longer from diagnosis of erythroderma (median survival, 6.2 yr) than did patients on other regimens (median survival, 3.05 yr). The addition of x-ray or chemotherapeutic programs did not increase significant long-term benefit. Seven patients receiving chlorambucil and prednisone had remission (1 yr or more); most patients had partial remission, but two patients did not have a response to this program. The complications of sepsis, progressive disease, and lymphoma occurred both in patients who were and in those who were not treated with chlorambucil and prednisone. We believe that a regimen of low-dose chlorambucil and prednisone is satisfactory treatment that can be the basis for comparison of new therapeutic approaches to Sézary syndrome.