Cancerbackup: Q-1080313958

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My wife has been told that she has SÈzary syndrome. What will that mean for her?

Lymphomas are cancers of the lymphoid tissue, which is part of our body's immune system. One of the most important cells in our immune system is a type of white blood cell called a lymphocyte. There are two types of lymphocytes: 'B-cells' and 'T-cells'. All lymphocytes are produced in the bone marrow and start life as young, immature cells called stem cells. Some lymphocytes continue their development in the bone marrow or lymph nodes and these are called B-cells but others move to the thymus gland and they are called T-cells. The description and classification of the various types of non-Hodgkin lymphoma has developed over the years as more has been learnt both about the immune system and the cancers themselves. The most recent classification divides non-Hodgkin lymphoma into some fifteen different tumour types. Each of these will have different patterns of behaviour and may need different approaches to treatment.

SÈzary syndrome is a T-cell lymphoma that is very similar to mycosis fungoides. It is a rare disease that only occurs in adults. Most types of lymphoma usually appear as painless swellings in the neck, armpit or groin, caused by enlarged lymph nodes. SÈzary syndrome is different in that it is a widespread disease affecting the skin, lymph nodes and blood. In its later stages, many organs may be affected by it. Patients usually present with swelling of lymph nodes and skin changes. These can be quite florid with generalised redness of the skin  - called ‘erythroderma’. It may also develop from mycosis fungoides where the skin lesions are more localised. Other problems people report are hair loss, itching and problems with nail growth.

The precise symptoms caused by the cancer will vary with which organs are affected but some general symptoms that may be present include night sweats, unexplained fever, loss of appetite, weight loss or tiredness. The diagnosis is made by a small operation, called a biopsy, which takes away a sample of the suspect lymphatic tissue to examine the cells under the microscope. There are also cells in the blood that have a typical appearance under the microscope and a typical pattern of markers on the surface that can be detected by special tests. Additional tests, including blood tests, x-rays, scans and bone marrow samples, are then used to get more information about the lymphoma and to see if it has spread elsewhere in the body. This is important in deciding treatment. Several treatments have been used in this condition and your doctor can outline the likely risks and benefits of each for your wife’s unique case. Unfortunately in most people, this is an aggressive disease with only limited numbers surviving beyond 5 years.


Content last reviewed: 01 August 2004
Page last modified: 08 June 2006

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