1. Sézary Syndrome Defined
Sézary Syndrome is a rare variation of Non-Hodgkin's Lymphoma, which is cancer of the white blood cells. Sézary Syndrome can develop from CTCL, or cutaneous t-cell lymphoma. CTCL is generally limited to the skin and is slow growing. In CTCL, the t-cells become cancerous and affect only the skin, called mycosis fungoides. The syndrome is a rare variation of CTCL, in which the blood can also be affected by the malignant t-cells and is considered an advanced form of mycosis fungoides. Mycosis fungoides do not necessarily progress into Sézary Syndrome.
2. Symptoms That Define Sézary Syndrome
The main symptom of Sézary Syndrome is a red rash that is widespread and covers most of the body, including clothing protected areas. The skin becomes scaly and itchy. There may be areas of the skin that are thickened or patchy as well. Some people with Sézary Syndrome may have lumps or tumors on their skin. The skin can also peel and become painful.
3. Diagnosis May Be Difficult
CTCL may be difficult to diagnose because it is slow growing. It often may be misdiagnosed as psoriasis or eczema because the symptoms are similar. Many people diagnosed with CTCL have had it for many months or years before the symptoms progress enough to be diagnosed. The initial symptoms of Sézary Syndrome make it easier to diagnose than CTCL that hasn't progressed to the stage of the syndrome. Your doctor will take a history of the symptoms and order a blood test to check the number of red blood cells, white blood cells, hemoglobin and platelets. A biopsy will be done to verify malignant cells. The doctor will order an immunophenotyping test, in which suspect cells are compared to normal cells to identify certain cell markers for a definitive diagnosis.
4. Treatments and Clinical Trials
Electron beam radiation is often used to treat early stages of CTCL. The electron beam treats only the skin and doesn't penetrate deeper into the organs. Light therapy, using ultraviolet light is also used to treat t-cell lymphoma of the skin. Special creams have chemotheraputic agents added to them and help some patients with CTCL. If the lymphoma has progressed to the lymph glands, internal organs or blood, then regular chemotherapy is used to treat lymphoma of the skin. Patients with Sézary Syndrome are often referred to clinical trials because it's a rare disease and treatments vary widely. Patients with Sézary Syndrome are given medications to treat the symptoms and some are given systemic chemotherapy.
5. Follow-ups and Prognosis
After receiving a diagnosis of Sézary Syndrome, patients are urged to continue with regular medical check-ups. Lymphoma can progress but it may present with symptoms of infection, like a virus or cold. These symptoms are confusing and they need to be checked by the doctor to make sure the cancer isn't recurring. Because most CTCL are slow growing cancers, patients can live a fairly normal life for many years with the disease.
