What to Know About Sézary Syndrome

Medically Reviewed by Jabeen Begum, MD on November 24, 2021
4 min read

Sézary syndrome is a rare form of lymphoma. It’s an aggressive type of cancer and progresses quickly.

Sézary syndrome is a rare type of cutaneous T-cell lymphoma (CTCL). This is a group of disorders that occur when your T-cells become cancerous. These cells are known as Sézary cells.

T-cells are a type of white blood cell or lymphocyte. They develop from stem cells in your bone marrow and are part of your immune system.

Cutaneous T-cell lymphoma causes your T-cells to multiply out of control. For people with Sézary syndrome, these cells can become cancerous, spreading from your skin to your blood. The cancer may also spread to other parts of your body, like your lymph nodes. 

Sézary syndrome is quite rare. Each year in the U.S., there are about 3,000 new cases of cutaneous T-cell lymphoma, about 15% of which are Sézary syndrome. 

The disease can affect anyone, but it is more common in people who ages 50 and older. It’s slightly more common in men than women.

Doctors don’t know the exact cause of this disease, but a small number of people with Sézary syndrome have human T-lymphotropic viruses type 1 and type 2.

This virus is passed from one person to another through direct contact with infected bodily fluids. This includes breast milk, blood, and semen. Most people with this virus don’t have symptoms, but researchers think that there may be a link to some diseases like T-cell lymphoma.

People with Sézary syndrome usually have some chromosomal abnormalities in the cancerous T-cells. This changes the DNA of the cells. Experts don’t yet know whether these DNA changes play a role in how the disease develops.

Sézary syndrome can cause skin problems like:

  • Red rash over at least 80% of your body
  • Itchy, dry, or peeling skin
  • Thickened skin on your palms and soles
  • Skin tumors 

You may have other symptoms like:

  • Swelling in your arms and legs because of fluid buildup (edema)
  • Hair loss (alopecia)
  • Abnormal nail growth
  • Fever
  • Enlarged liver
  • Enlarged spleen
  • Unexplained weight loss
  • Lower eyelids turning outward, away from your eye (ectropion)
  • Swollen lymph nodes
  • Problems controlling your body temperature

Sézary syndrome is a rare disease, so, usually, a hematopathologist or a dermatopathologist is needed to confirm the diagnosis.

As Sézary syndrome is a type of cutaneous T-cell lymphoma, many of the same diagnostic tests are used. These include: 

  • A physical exam and medical history
  • Blood tests to identify markers (antigens) on the surface of cells in your blood
  • Lymph node biopsy or skin biopsy. A small piece of tissue is removed and examined in a lab.
  • Imaging tests to see if the cancer has spread to other organs or lymph nodes. These tests include magnetic resonance imaging (MRI), computerized tomography (CT), and positron emission tomography (PET) scans.
  • For some people, a bone marrow biopsy may be done to check what stage the disease is at. 

Sézary syndrome spreads fast. Treatment can help you manage your symptoms, but there’s no cure. Treatment for this condition includes:

Immunotherapy. This is also known as biological therapy. It stimulates your immune system to fight cancer. 

Extracorporeal photopheresis (ECP). This treatment removes blood from your body using a machine. The Sézary cells are treated with a drug that makes them light sensitive. Ultraviolet light is used to kill the cancerous cells. Your blood is then returned to your body. 

Ultraviolet phototherapy: Your skin is exposed to ultraviolet light in the A range (UVA) or B range (UVB). PUVA is a combination treatment that’s also used. You’ll take a drug called psoralen before being exposed to ultraviolet A phototherapy.

Targeted therapy. This treatment uses drugs to target certain genes or proteins that are involved in the growth and survival of cancer cells.

Radiation therapy. Total skin electron beam radiation therapy delivers high doses of radiation to your skin to kill cancer cells.

Chemotherapy. For Sézary syndrome, chemotherapy may be done on your skin or throughout your body. This may be done through an intravenous drip or a pill. 

Other drug therapies. To stop cancer cells from dividing, your doctor may recommend histone deacetylase inhibitors. Retinoid gels or creams may slow the growth of cancer cells. Corticosteroid creams or ointments can relieve your irritated skin. 

Sézary syndrome is an aggressive cancer. Only about quarter of people with this disease are still alive 5 years after developing this condition. Newer treatments have helped improve survival rates, though.