What is Sézary syndrome?

Sézary syndrome is a rare, aggressive type of cutaneous T-cell lymphoma in which lymphocytes become cancerous and affect the skin.

The cancerous T cells — known as Sézary cells — appear in the skin, lymph nodes and blood of people with the condition. The cells can rapidly spread to the lymph nodes, liver, spleen and bone marrow, placing people with Sézary syndrome at increased risk for another lymphoma or other cancer type. Sézary syndrome is the second most common type of T-cell lymphoma, and it accounts for nearly 3% to 5% of cutaneous T-cell lymphomas.

Risk factors and symptoms

Sézary syndrome effects adults aged older than 60 years. Although the exact cause is unknown, people with Sézary syndrome have one or more chromosomal abnormalities that occur during a lifetime that are discovered in the DNA of cancerous cells.

Sézary cells are found in erythroderma — a red, severely itchy rash — that covers significant areas of the body. These cells are characterized by an abnormally shaped nucleus.

The skin cells themselves are not cancerous; rather, the rash appears when Sézary cells move from the blood into the skin, according to the NIH.

Other symptoms of Sézary syndrome include enlarged lymph nodes, alopecia, thickened skin on the palms of the hands and soles of the feet, disfigured fingernails and toenails, and outward turned lower eyelids. In some cases, people with Sézary syndrome are not able to control their body temperature.

Treatment

Sézary syndrome is detected by tests that examine the skin and blood. Prognosis and treatment options depend upon factors such as cancer stage and lesion type.

Recent advancements in treatment have extended survival to the point where patients with early-stage disease may live for many years.

Treatment for Sézary syndrome is most often palliative, and it includes biologic therapy, chemotherapy, photodynamic therapy, radiation therapy and targeted therapy. Patients may also undergo PUVA therapy (photochemotherapy) with or without systemic chemotherapy or biologic therapy.

Looking ahead

Various new types of treatments are being examined in clinical trials. These include ultraviolet B radiation therapy, high-dose chemotherapy and radiation therapy with stem cell transplant.

Ultraviolet B radiation therapy involves a special lamp or laser that targets the skin with UVB radiation. During high-dose chemotherapy and radiation therapy with stem cell transplant, patients receive significant doses of chemotherapy and radiation therapy in addition to the replenishment of blood-forming cells that are depleted by cancer treatment.

More information is available at the following websites:

www.cancer.gov/types/lymphoma/patient/mycosis-fungoides-treatment-pdq

ghr.nlm.nih.gov/condition/sezary-syndrome

http://www.clfoundation.org/online-learning-center/disease/ss/s-zary-syndrome-ss