Sezary Syndrome

You have been diagnosed with a condition called Sezary syndrome which is a type of skin lymphoma. In the north east of England we have a specialist clinic for patients with this rare type of skin cancer which is held on a monthly basis in Newcastle upon Tyne. This information sheet provides a summary of the condition and provides links to other information and local service contacts.

What is skin lymphoma?

Lymphoma is a cancer that begins in cells called ‘lymphocytes’ which are a vital part of our immune system. These cells are usually found in blood and lymph nodes (glands) but also in other parts of the body such as the skin, gut and spleen.

A lymphoma develops if lymphocytes grow out of control or don’t die off after their normal life span. These lymphocytes can build up and form a cancerous collection of cells. Cutaneous skin lymphoma develops when the lymphoma starts in the lymphocytes in the skin (‘cutaneous’ means anything related to the skin). This does not usually affect the internal organs.

There are many different types of lymphoma but they can be divided into two main groups, Hodgkin and Non-Hodgkin lymphoma. These differ in how they develop, how they behave and in how they are treated. Skin lymphomas are a type of Non-Hodgkin lymphoma.

Lymphomas starting in the skin account for around 1 in 40 of all Non-Hodgkin lymphomas and these primary ‘skin lymphomas’ are therefore quite rare. If a lymphoma starts somewhere else in the body such as the lymph nodes, then spreads to the skin, it is not considered to be a skin lymphoma.

Skin lymphomas can develop from either of the two types of lymphocytes in the body; these are called T lymphocytes (T-cells) or B Lymphocytes (B-cells). Most skin lymphomas develop from T-cells and are known as primary cutaneous T-cell lymphoma or CTCL for short. Mycosis Fungoides (which appears as reddish skin patches) is the most common type of CTCL and can progress over many years. Sezary syndrome is an advanced form of this.

Why do I have skin lymphoma?

The cause of skin lymphoma is unknown. It can occur in anyone, but is more common in men, people whose immune system isn’t working properly (e.g. taking medicines which suppress it) and older people. It cannot be inherited and cannot be caught or passed to other people. Nothing you have done will have caused the lymphoma.

How is skin lymphoma diagnosed?

Many skin lymphomas look very similar to common skin conditions such as eczema or psoriasis, and making the diagnosis is often not straightforward. Many develop very slowly, some taking up to 40 years. It can therefore be months or even years before you are referred to a dermatologist and the condition is diagnosed. Such a delay is unlikely to affect the outcome of your disease.

Skin lymphoma is diagnosed by a skin biopsy. However the diagnosis is not always easy and sometimes patients need more than one biopsy over a period of time before the diagnosis can be established.

What tests do I need to have?

Skin lymphomas are normally assessed by examination of the skin, feeling for lymph glands, routine blood tests and the results of the skin biopsy. Imaging tests such as CT (Computed Tomography), MRI (Magnetic Resonance Imaging) and/or PET (Positron Emission Tomography) scans may also be requested to determine if the cancer has spread to lymph nodes or other organs and a bone marrow biopsy may be necessary to verify complete staging.

What do the ‘stages’ mean?

The stage of the lymphoma is an indication of how much the lymphoma is affecting the body so because mycosis fungoides and sezary syndrome are such rare cancers, it is important to confirm diagnosis by a dermatopathologist.

There are four stages of skin lymphoma; 1 to 4, although these are further broken down into A and B for each stage. Stages 1A, 1B, and 2A are considered early stage disease and 2B, 3 and 4 more advanced.

Stage 1A

Less than 10% of skin is covered in red patches and/or plaques.

Stage 1B

10% or more of the skin is covered in patches and/or plaques.

Stage 2A

Any amount of the skin surface is covered with patches and/or plaques; lymph nodes are enlarged, but the cancer has not spread to them.

Stage 2B

One or more tumours are found on the skin; lymph nodes may be enlarged, but the cancer has not spread to them.

Stage 3

Nearly all of the skin is reddened and may have patches, plaques or tumours; lymph nodes may be enlarged, but the cancer has not spread to them.

Stage 4A (Sezary syndrome)

Most of the skin area is reddened and there is involvement of the blood with malignant cells or any amount of the skin surface is covered with patches, plaques or tumours; cancer has spread to the lymph nodes and the lymph nodes may be enlarged.

Stage 4B

Most of the skin is reddened or any amount of the skin surface is covered with patches, plaques or tumours; cancer has spread to other organs and lymph nodes may be enlarged whether cancer has spread to them or not.

What is the prognosis for skin lymphoma?

The outlook (prognosis) for Sezary syndrome sufferers is variable and whilst there is no cure, treatment aims to keep the condition under good control. If you want more detailed information, please ask your consultant responsible for your care.

How is Sezary syndrome treated?

There are several effective therapies available to treat Sezary syndrome. Because the disease is systemic (affecting the entire body), Sezary syndrome is usually not treated with skin therapies alone. The specific treatment for individual patients is based on a variety of factors, including the patient’s general health and stage of disease.

There are several types of standard treatment for Sezary syndrome:

Phototherapy: PUVA (ultraviolet-A light is directed onto the skin and the patient is given the drug psoralen); UVB (skin directed ultraviolet-B light); NBUVB (skin directed narrow band ultraviolet-B light).
Biologic or immunotherapy therapy is a treatment used to stimulate a patient’s own immune system to fight the cancer.
Retinoids, which are drugs related to vitamin A and can slow certain types of cancer cells (examples are: Interferon alpha and Bexarotene).
ECP (extracorporeal photophersis), a procedure used to expose the blood to ultraviolet light.
Radiation therapy, which uses high energy X-rays or other types of radiation to kill cancer cells or keep them from growing.
Chemotherapy, a drug given either orally or through an infusion in a vein to stop the growth of rapidly dividing cancer cells.

Further information on specific treatments or your possible involvement in a clinical trial can be discussed with you and written information leaflets provided.

Follow-up and contact details

You will be offered follow up in the long term in the CTCL clinic in Newcastle upon Tyne. If you are concerned or your condition changes between appointments then please contact us for an earlier appointment by contacting the consultant dermatology secretaries on 0191 2824340 or specialist nurse team on 0191 2824110.