Sezary syndrome is a form of non-Hodgkin's lymphoma. In this form, white blood cells become cancerous and affect both the skin and the peripheral blood. An advanced type of mycosis fungoides, Sezary syndrome is considered a cutaneous T-cell lymphoma because when this condition is present, the cancer has already spread throughout the blood system and is having a visible effect on the skin. Lymphomas of all types primarily attack the immune system and compromise the body's ability to fight off infection.
Mycosis fungoides refers to a condition where the T-cell lymphocytes of the blood become cancerous. In a healthy body, T-cell lymphocytes are cells in the bloodstream that control the immune system's responses to invading cells and that actively attack these foreign bodies as well as cells harboring a virus or cancer cells. When these cells become compromised themselves, the body's ability to fight illness and disease is vastly diminished.
The earliest sign of Sezary syndrome is a red rash on the skin, typically in places that are not exposed to the sun or the elements. The skin might become scaly, and this is not usually accompanied by any other symptoms. As the disease progresses, the rash will become thin and can take on an appearance similar to eczema. Red patches will then become thicker, and in the most severe stages of the disease, tumors can form on the skin. Throughout the progression of the disease, the skin is itchy and painful, often peeling in patches that can become infected.
Not all cases of mycosis fungoides develop into Sezary syndrome. In order to diagnose the disease, a physical exam checking for lesions, rashes and any other unusual skin conditions can be performed. In addition, complete blood work can also be run to determine whether the levels of red and white blood cells are normal. If abnormalities do show, the doctor might perform a biopsy to check for cancerous tissue or a might perform a blood smear to examine blood under a microscope and see whether there are any unusual cells. Biopsies of skin cells can also be examined to see whether there are any cutaneous abnormalities.
Sezary syndrome can be difficult to cure, but patients with a Sezary diagnosis can live for years with the disease. Depending on how far the cancer has spread, whether it has reached the lymph nodes, the types of lesions and their locations as well as the number of T-cells in the blood, most patients receive some form of palliative care. This will ease the effects of the symptoms.
