Sézary Syndrome: Symptoms, Staging, and More

You might also hear Sézary syndrome called Sézary erythroderma or Sézary’s lymphoma.

The hallmark sign of Sézary syndrome is erythroderma, a discolored, itchy rash that can eventually cover the majority of the body. Other signs and symptoms include:

• swelling of the skin
• skin plaques
• enlarged lymph nodes
• thickening of skin on the palms and soles
• abnormalities of fingernails and toenails
• lower eyelids that turn outward
• hair loss
• trouble regulating body temperature

Sézary syndrome can also cause an enlarged spleen or problems with the lungs, liver, and gastrointestinal tract.

The exact cause isn’t clear. But most people with Sézary syndrome have chromosomal abnormalities in the DNA of the cancerous cells, but not in healthy cells.

These aren’t inherited irregularities, but changes that happen over a lifetime. Sézary syndrome is most common in people over the age of 60. The rate of T-cell lymphoma diagnoses is also increasing, with incidence rates highest in males and African Americans.

The most common abnormalities are DNA loss from chromosomes 10 and 17 or DNA additions to chromosomes 8 and 17. Still, it’s not certain that these abnormalities cause the cancer.

A physical examination of your skin may alert the doctor to the possibility of Sézary syndrome. Diagnostic testing may include blood tests to identify markers (antigens) on the surface of the cells in the blood.

As with other cancers, a biopsy is the best way to reach a diagnosis. For a biopsy, the doctor will take a small sample of skin tissue. A pathologist will examine the sample under a microscope to look for cancer cells.

Doctors may also order lymph node and bone marrow biopsies. Imaging tests, such as CT, MRI, or PET scans, can also help determine if cancer has spread to the lymph nodes or other organs.

Staging

Staging tells how far the cancer has spread and what the best treatment options are. Sézary syndrome is staged as follows, according to the National Cancer Institute:

• 1A. Less than 10 percent of the skin is covered in red patches or plaques.
• 1B. More than 10 percent of the skin is red.
• 2A. Any amount of skin is involved. Lymph nodes are enlarged, but not cancerous.
• 2B. One or more tumors larger than 1 centimeter have formed on the skin. Lymph nodes are enlarged, but not cancerous.
• 3A. Most of the skin is red and may have tumors, plaques, or patches. Lymph nodes are normal or enlarged, but not cancerous. The blood may or may not contain a few Sézary cells.
• 3B. There are lesions over most of the skin. Lymph nodes may or may not be enlarged. The number of Sézary cells in the blood is low.
• 4A(1). Skin lesions cover any part of the skin surface. Lymph nodes may or may not be enlarged. The number of Sézary cells in the blood is high.
• 4A(2). Skin lesions cover any part of the skin surface. There are enlarged lymph nodes and the cells look very abnormal under microscopic examination. Sézary cells may or may not be in the blood.
• 4B. Skin lesions cover any part of the skin surface. The lymph nodes may be normal or abnormal. Sézary cells may or may not be in the blood. Lymphoma cells have spread to other organs or tissues.

A number of factors influence which treatment may be best for you. Among them are:

• stage at diagnosis
• age
• other health problems

The following are some of the treatments for Sézary syndrome.

Psoralen and ultraviolet A (PUVA) therapy

In this type of therapy, a doctor will use psoralen, an injectable drug, and ultraviolet light to destroy cancer cells.

This drug collects more in cancer cells than in normal cells, and only activates when a medical professional directs ultraviolet A (UVA) light at your skin. Once these drugs are active they work to destroy the cancer cells they collect with minimal damage to healthy tissue.

Extracorporeal photochemotherapy/photopheresis (ECP)

This form of therapy also uses a combination of drugs and UV light to destroy cancer cells.

However, during this treatment, a doctor will remove blood from your body and treat it with light-reactive drugs and UVA light. Once treatment is complete the doctor will then reinfuse this blood back into your bloodstream.

Radiation therapy

In radiation therapy, medical professionals use high-energy X-rays to destroy cancer cells. In external beam radiation, a machine sends rays to targeted areas of your body.

Radiation therapy can relieve pain and other symptoms as well. Total skin electron beam (TSEB) radiation therapy uses an external radiation machine to aim electrons at the skin of your whole body.

You can also have UVA and ultraviolet B (UVB) radiation therapy using a special light aimed at your skin.

Learn more about UVA and UVB rays.

Chemotherapy

Chemotherapy is a systemic treatment in which powerful drugs are used to kill cancer cells or stop their division. Some chemotherapy drugs are available in pill form, and others must be given intravenously.

Immunotherapy (biologic therapy)

Drugs such as interferons are used to prompt your own immune system to fight cancer.

Drugs used to treat Sézary syndrome include:

• alemtuzumab (Campath), a monoclonal antibody
• bexarotene (Targretin), a retinoid
• brentuximab vedotin (Adcetris), an antibody-drug conjugate
• chlorambucil (Leukeran), a chemotherapy drug
• corticosteroids to relieve skin symptoms
• cyclophosphamide (Cytoxan), a chemotherapy drug
• denileukin diftitox (Ontak), a biologic response modifier
• gemcitabine (Gemzar), an antimetabolite chemotherapy
• interferon alfa or interleukin-2, immune stimulants
• lenalidomide (Revlimid), an angiogenesis inhibitor
• liposomal doxorubicin (Doxil), a chemotherapy drug
• methotrexate (Trexall), an antimetabolite chemotherapy
• pentostatin (Nipent), an antimetabolite chemotherapy
• romidepsin (Istodax), a histone deacetylase inhibitor
• vorinostat (Zolinza), a histone deacetylase inhibitor

Your doctor may prescribe combinations of drugs or drugs plus other therapies. This will be based on the stage of Sézary syndrome and how well you respond to a particular treatment.

Treatment for stage 1 and 2 Sézary syndrome are likely to include:

• topical corticosteroids
• retinoids, lenalidomide, histone deacetylase inhibitors
• PUVA
• radiation with TSEB or UVB
• biologic therapy by itself or with skin therapy
• topical chemotherapy
• systemic chemotherapy, possibly combined with skin therapy

Stages 3 and 4 may be treated with:

• topical corticosteroids
• lenalidomide, bexarotene, histone deacetylase inhibitors
• PUVA
• ECP alone or with TSEB
• radiation with TSEB or UVB and UVA radiation
• biologic therapy by itself or with skin therapy
• topical chemotherapy
• systemic chemotherapy, possibly combined with skin therapy

In cases of recurrent Sézary syndrome, a stem cell transplant may be an option.

Sézary syndrome is an aggressive form of cancer. Sézary cells can spread to your lymph nodes and other organs including the spleen, liver, and bone marrow. People with Sézary syndrome have a higher risk of developing other forms of cancer.

As the syndrome can cause discomfort across large portions of the skin, it may impact your quality of life. You may also be at an increased risk of skin infection if rashes cause breaks in your skin.

As the exact cause of Sézary syndrome is unknown, there are no sure ways to prevent its development.

Early diagnosis of the condition can improve your outlook. Seeking medical help if presenting with symptoms of Sézary syndrome can help to diagnose the condition in its earlier stages.

Sézary syndrome is a particularly aggressive cancer. With treatment, you may be able to slow disease progression or even go into remission. But a weakened immune system can leave you vulnerable to opportunistic infection and other cancers.

Historically, Average survival has been around 2 to 4 years after development of the syndrome, but this rate is improving with newer treatments.

See your doctor and start treatment as soon as possible to ensure the most favorable outlook.