Sezary Syndrome

Overview of Sezary syndrome (SS)

Sezary syndrome (SS) is a subtype of cutaneous T-cell lymphoma (CTCL).  Sezary syndrome (SS) is a very rare and often aggressive (fast growing) lymphoma. It accounts for around 3-5% of all cutaneous (skin) T-cell lymphomas (CTCL).

Sezary syndrome (SS) is like mycosis fungoides (MF) but it affects the blood as well as the skin. Often large areas of skin or lymph nodes are affected and Sezary syndrome tends to follow an aggressive (fast growing) course. Patients can develop it without having any milder form of skin lymphoma before.

Why is it called Sezary syndrome?

It is called ‘Sezary syndrome’ as there is the presence of cancerous T-cells called ‘Sezary cells’.  These cells are found in the blood and abnormally enlarged lymph nodes.

Who is affected by Sezary syndrome (SS)

Although Sezary syndrome can affect all ages, it is most commonly diagnosed in adults over 60 years of age. It is slightly more common in men than women.

The exact cause is unknown. However, a small percentage of cases are associated with human T-lymphotropic viruses (HTLV) type 1 and type 2. 

Symptoms of Sezary syndrome (SS)

Some of the common symptoms of sezary syndrome can include:

• Large areas of bright red, thickened, swollen and sore skin (erythroderma) that sometimes peels
• Large numbers of abnormal T cells called ‘Sezary cells’ in the blood
• Severe itching
• Swollen lymph nodes
• Hair loss
• Thickening of the skin on the palms and on the soles of the feet
• Thickening of nails
• Drooping of the lower eyelid (ectropion)
• Hepatosplenomegaly
• Fever
• Weight loss

Diagnosis of Sezary syndrome

A skin and/or lymph node biopsy is needed to confirm a diagnosis of Sezary syndrome.  A biopsy is a procedure to remove a small piece of tissue to look at it under the microscope by a pathologist (a specialist doctor) to see what the cells look like.  The biopsy can be done under local or general anaesthetic depending on what part of the body is being biopsied.

Sezary syndrome is very rare and may often need to be diagnosed by a specialist team who are experts in lymphoma. 

Waiting for results can be a difficult time and it may help to talk to family, friends or a specialist nurse.  

Staging of Sezary syndrome (SS)

Once a diagnosis of Sezary syndrome is made, further tests are required to see where else in the body the lymphoma has affected or located.  This is called staging.  The staging of lymphoma helps the doctor to know what’s the best treatment for the patient. 

There are four stages from stage 1 (lymphoma in one area) through to stage 4 (lymphoma that is widespread). 

To find out the stage of Sezary syndrome (SS) the doctor checks:

• How the skin is affected-plaques, patches or tumours
• How much of the skin is affected
• Whether the lymphoma is in the lymph nodes
• Whether the lymphoma is in any organs

Other examination and tests may include:

• Physical examination and patient history
• Blood tests to identify antigens, or marker on the surface of the cells in the blood
• PET (positron emission tomography) scan
• CT (computerised tomography)
• Bone marrow biopsy (for some people)

Early stage disease

Early stage is anything up to 2A. Most patients have this stage of skin lymphoma when diagnosed.

Stage 1:

●      1A means less than 10% of the skin surface is affected

●      1B means that more than 10% of the skin surface is affected (roughly size of both arms)

Stage 2:

●      2A means there are patches or plaques on the skin and the lymph nodes are enlarged but they do not contain abnormal lymphoma cells.

●      2B means that there are one or more raised lumps or tumours in the skin and the lymph nodes may or may not be enlarged but do not contain lymphoma cells

Advanced stage disease

Stage 3:

●      80% or more of the skin is affected, with generalised redness, swelling, itching and sometimes pain.  The lymph nodes are enlarged but do not contain abnormal lymphoma cells.

●      3A means there are a few or no lymphoma cells in the bloodstream

●      3B means there are moderate numbers of lymphoma cells in the bloodstream (Sezary syndrome)

Stage 4:

●      Stage 4 mycosis fungoides or sezary syndrome affects the skin and has also spread to the bloodstream, lymph nodes or other organs.

●      4A means there are numerous abnormal lymphoma cells in the bloodstream, or the lymph nodes contain lymphoma cells.

●      4B means the lymphoma is in other organs

Prognosis of Sezary syndrome (SS)

Treatment for Sezary syndrome is systemic treatment such as biologic, immunotherapy or chemotherapy.  This disease is usually aggressive and patients may respond to treatment initially but relapse soon after.  Patients can suffer a number of serious infections , as their immune system is very compromised due to the lymphoma.  Skin is one of our main protective barriers to infection.  

Treatment for Sezary syndrome (SS)

Sezary syndrome affects the whole body. It needs to be treated with systemic treatment and the first choice of treatment is extracorporeal photopheresis (ECP). This treatment is where the white blood cells are removed from the body, a drug called psoralen is added to the white blood cells, this makes them light sensitive, the cells are then exposed to UV light and the treated white blood cells are then returned to the body. This might be combined with other treatments, such as:

• Methotrexate 
• Bexarotene
• Interferon-alfa

If Sezary syndrome does not respond to the initial treatment or has spread to other organs, other treatments can include:

• Phototherapy (UV light radiation directed to the skin) – with a drug
• Radiotherapy
• Chemotherapy
• Antibody therapy; alemtuzumab, brentuximab vedotin or mogamulizumab
• Young, fit patients with Sezary syndrome who have not responded to other treatments might have an allogeneic stem cell transplant (ASCT)
• Clinical trial participation

Living with Sezary syndrome (SS)

Most patients learn to manage their condition together with their specialist team. They generally use topical treatments in addition to systemic treatments if this is needed and some patients will use baths or other agents to reduce the infective load on their skin, e.g.  as bleach baths. Patients may also require wound care based on what their skin is doing at the time. Skin infections and skin pain often need managing by the medical team and as a general principle chemotherapy is reserved for late stage refractory disease.