Pre-Sézary syndrome.
Eighteen patients with erythroderma, recurrent cycles of circulating Sézary cells of less than 1,000 cells/mm3, and a chronic course were followed for a mean time of nearly 5 years and were diagnosed as having pre-Sézary syndrome. Only one patient died, and none developed lymphoproliferative disease. All ten patients who underwent patch testing showed positive results. The elevation of IgE was striking when this group was compared with a group with Sézary syndrome. Most patients achieved partial or complete remission on low-dose chlorambucil and prednisone therapy. Some patients had lymphocytic or lymphomatoid bands on skin biopsy specimens and were like previously reported patients with pre-Sézary syndrome whose condition progressed to Sézary syndrome. A nontoxic chemotherapy or an anti-T cell treatment program can control this chronic erythroderma state.
D000328:Adult; D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002908:Chronic Disease; D003348:Cortisone / Q000627:therapeutic use; D003873:Dermatitis, Exfoliative / Q000150:complications* / Q000175:diagnosis; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011701:PUVA Therapy; D010328:Patch Tests; D012751:Sezary Syndrome / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D013994:Tilorone / Q000627:therapeutic use
D000970:Antineoplastic Agents; D013994:Tilorone; D003348:Cortisone