SEZARY SYNDROME

Friday, February 20, 2009

Background

This Sezary cell is the malignant pleomorphic T cell seen in mycosis fungoides and has a convoluted nucleus. In the peripheral smear, these cells number greater than 1.0x10*9/l. Prior to sophisticated molecular techniques such as flow cytometry, a Sezary prep was performed by concentrating the white blood cells and doing a morphologic count. However, there are many inflammatory conditions which may elicit Sezary-like cells in the peripheral smear. In addition, a morphological appraisal of a peripheral smear slide is not a very sensitive technique. There are no known causes of Sezary syndrome. It can affect many organs. In early stage disease, the skin is the only organ affected; however, later stage disease can affect other organ systems. There are many different types of primary cutaneous lymphomas but they can be broadly divided into two categories, cutaneous T-cell lymphomas and cutaneous B-cell lymphomas. It is is characterized by skin abnormalities, extreme itching, enlarged lymph glands, and abnormal blood cells. Men appear to be affected more often than women, and black males appear to be at higher risk of developing the syndrome than white males. There are multiple therapies for Sézary syndrome. However, unless the disease is in an early stage, the chances for a complete cure are small.

Symptoms of Sezary syndrome
Some sign and symptoms related to Sezary syndrome are as follows:
• The skin may itch, and dry.
• Harmless swelling.
• Dark patches may develop on the skin.
• Red rash on the skin.
• Hair loss, in the areas of and surrounding the patches.
• Skin changes.
• Enlarged lymph glands in the neck.

How to Treat Sezary Syndrome
Sezary Syndrome is a variant of mycosis fungoides that occurs in about 5 percent of those cases. It is identified in patients already diagnosed with mycosis fungoides that have significant signs of malignant T-cell clones in their blood. These signs include having lymphocytes cerebriform nuclei in the blood at a concentration greater than 1000/mm^3. The following steps will show how to treat Sezary Syndrome.

Step1
Treat Sezary Syndrome on an outpatient basis in most cases. Symptomatic treatments such as emollients and anti-itch medication are combined with specific systemic and topical treatments.

Step2
Provide topical treatments only to Stage I patients in most cases of Sezary Syndrome. These include steroids, retinoids, chemotherapy (nitrogen mustard or bischloroethylnitrosourea) and ultraviolet radiation (UV-A and UV-B). Total body electron beam radiation also may be performed for Sezary Syndrome patients in the patch phase. These treatments are combined with systemic modalities if Sezary Syndrome is in the tumor phase.

Step3
Use systemic treatments by themselves or with topical medication. This therapy is indicated for patients in Stage IIB or greater, or Stage I patients not responsive or intolerant to topical treatments. Systemic treatments include extracorporeal photopheresis, fusion toxin treatment, oral retinoids and recombinant alfa interferon.

Step4
Determine sequential therapies based on availability, convenience and toxicity profiles. The major cause of death in Sezary Syndrome patients is infection so cellular immune function should be preserved as much as possible.

Step5
Consider less common treatments. Allogenic bone marrow transplants are still considered experimental for the treatment of Sezary Syndrome and combination chemotherapy is rarely used because of the complications from infections and limited improvement in response.

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