Abstract

Sézary disease is a type of cutaneous lymphoma that was first described by Albert Sézary. The affected cells are T-cells (so it is a T-cell lymphoma) that have pathological quantities of mucopolysaccharides. Sézary disease is sometimes considered a late stage of mycosis fungoides with lymphadenopathy. There are currently no known causes of Sézary disease.

Signs and symptoms

Sézary syndrome and mycosis fungoides are T-cell lymphomas whose primary manifestation is in the skin. The disease's origin is a peripheral CD4+ T-lymphocyte, although rarer CD8+/CD4- cases have been observed. Epidermotropism by neoplastic CD4+ lymphocytes with the formation of Pautrier's microabscesses is the hallmark sign of the disease. The dominant symptoms of the disease are:

1. Generalized erythroderma

2. Lymphadenopathy

3. Atypical T-cells ("Sézary cells") in the peripheral blood

4. Hepatosplenomegaly

5. Palmoplantar keratoderma

Diagnosis

Those who have Sézary disease often present with skin lesions that do not heal with normal medication. A blood test generally reveals any change in the levels of lymphocytes in the blood, which is often associated with a cutaneous T-cell lymphoma. Finally, a biopsy of a skin lesion can be performed to rule out any other causes.

The immunohistochemical features are very similar to those presented in mycosis fungoides except for the following differences:

1. More monotonous cellular infiltrates (large, clustered atypical pagetoid cells) in Sézary syndrome

2. Sometimes absent epidermotropism

3. Increased lymph node involvement with infiltrates of Sézary syndrome.

Treatment

Treatment typically includes some combination of photodynamic therapy, radiation therapy, chemotherapy, and biologic therapy.

Treatments are often used in combination with phototherapy and chemotherapy, though pure chemotherapy is rarely used today. No single treatment type has revealed clear-cut benefits in comparison to others, treatment for all cases remains problematic.

Treatment | Radiation therapy

A number of types of radiation therapy may be used including total skin electron therapy. While this therapy does not generally result in systemic toxic effects it can produce side effects involving the skin. It is only avaliable at a few institutions.

Treatment | Chemotherapy

Romidepsin, vorinostat and a few others are a second-line drug for cutaneous T-cell lymphoma. Mogamulizumab has been approved in Japan and waiting FDA approval in the United States. There are dozens of clinical trials, with a few in Phase III.

Epidemiology

In the Western population, there are around 0.3 cases of Sézary syndrome per 100,000 people. Sézary disease is more common in males with a ratio of 2:1, and the mean age of diagnosis is between 55 and 60 years of age.