What is Sézary syndrome?
Sézary syndrome is an aggressive form of a type of blood cancer called cutaneous T-cell lymphoma. Cutaneous T-cell lymphomas occur when certain white blood cells, called T cells, become cancerous; these cancers characteristically affect the skin, causing different types of skin lesions.
Who discovered Sézary syndrome?
Sézary disease, or Sézary syndrome, is a type of cutaneous T-cell lymphoma that was first described by Albert Sézary. The affected T cells, known as Sézary’s cells or Lutzner cells, have pathological quantities of mucopolysaccharides.
What stage is Sézary syndrome?
When there is a high number of Sézary cells in the blood, the disease is called Sézary syndrome. Stage IV is divided into stages IVA1, IVA2, and IVB. In stage IVA1, patches, papules, plaques, or tumors may cover any amount of the skin surface, and 80% or more of the skin surface may be reddened.
What is characteristic morphology for Sézary cells?
Morphology. The Sézary cells have a variable amount of non-granular cytoplasm and show the characteristic delicately convoluted, cerebriform nucleus with condensed chromatin and inconspicuous nucleoli (Figures 48.8B inset and 48.8A). These cells may vary in size, with the smaller forms referred to as Lutzner cells.
How is Sézary syndrome diagnosed?
If your provider suspects Sézary syndrome, they may do the following tests:
- Biopsy: A healthcare provider does a biopsy of your skin, lymph nodes or bone marrow.
- Blood tests: A complete blood count and peripheral blood smear check your red and white blood cells, platelets and hemoglobin.
How is Sézary syndrome treated?
Lenalidomide is a drug that helps the immune system kill abnormal blood cells or cancer cells and may prevent the growth of new blood vessels that tumors need to grow. Vorinostat and romidepsin are two of the histone deacetylase (HDAC) inhibitors used to treat mycosis fungoides and Sézary syndrome.
How do you test for Sézary syndrome?
Blood tests allow doctors to measure the level of white blood cells in the body, which can determine whether you have Sézary syndrome. People with mycosis fungoides usually do not have cancerous T-cell lymphocytes circulating in the blood. When they do, it is a sign that the condition may be more advanced.
What is Sézary cells?
(sey-zah-REE sel) A cancerous T cell (a type of white blood cell) found in the blood, skin, and lymph nodes of patients who have a fast-growing type of skin lymphoma called Sézary syndrome. Under a microscope, Sézary cells have an abnormally shaped nucleus and look larger than normal T cells.
What are Sézary cells?
How long can you live with Sézary syndrome?
A recent meta-analysis including 6,279 patients with MF and Sezary syndrome found that about 10–20% of stage IB patients don’t survive 5 years, whereas patients with advanced-stage MF and Sezary syndrome have a 5-year survival chance of about 20–60%.
What is the difference between Sézary syndrome and mycosis fungoides?
Mycosis fungoides and Sézary syndrome are types of cutaneous T-cell lymphoma. A sign of mycosis fungoides is a red rash on the skin. In Sézary syndrome, cancerous T-cells are found in the blood. Tests that examine the skin and blood are used to diagnose mycosis fungoides and Sézary syndrome.
What does Sézary syndrome look like?
Most patients with Sézary syndrome have extensive red, itchy rash covering at least 80 percent of the body. In some cases, thicker, red patches (or plaques) and tumors may also appear. In addition, these symptoms may be accompanied by changes in the nails, hair, eyelids, and the presence of enlarged lymph nodes.