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Pre-Sézary syndrome.
Journal of the American Academy of Dermatology
|June 1, 1984
Summary
Pre-Sézary syndrome, characterized by erythroderma and low circulating Sézary cells, shows a favorable prognosis with effective treatment. Most patients achieve remission with chemotherapy, indicating manageable chronic erythroderma.
Area of Science:
- Dermatology
- Oncology
- Immunology
Background:
- Pre-Sézary syndrome is a chronic erythroderma condition.
- Patients exhibit recurrent Sézary cells (<1,000/mm³).
- It represents an early stage potentially preceding Sézary syndrome.
Purpose of the Study:
- To characterize the clinical course and treatment outcomes of pre-Sézary syndrome.
- To evaluate prognostic factors and therapeutic strategies.
Main Methods:
- Longitudinal follow-up of 18 patients diagnosed with pre-Sézary syndrome.
- Clinical assessment, including patch testing and IgE level measurement.
- Treatment with low-dose chlorambucil and prednisone, and anti-T cell therapies.
Main Results:
- A mean follow-up of nearly 5 years revealed a low mortality rate (1/18) and no progression to lymphoproliferative disease.
- Patch testing was positive in all 10 tested patients.
- Elevated IgE levels were observed compared to Sézary syndrome.
- Most patients achieved partial or complete remission with chemotherapy.
Conclusions:
- Pre-Sézary syndrome demonstrates a generally favorable prognosis.
- Non-toxic chemotherapy or anti-T cell treatments can effectively manage this chronic condition.
- Early intervention may prevent progression to Sézary syndrome.