Sézary Syndrome—Lymphoma

Also called: Non-Hodgkin lymphoma

Lymphoma is a cancer of a part of the immune system called the lymph system. There are many types of lymphoma. One type is Hodgkin disease. The rest are called non-Hodgkin lymphomas.

Non-Hodgkin lymphomas begin when a type of white blood cell, called a T cell or B cell, becomes abnormal. The cell divides again and again, making more and more abnormal cells. These abnormal cells can spread to almost any other part of the body. Most of the time, doctors don’t know why a person gets non-Hodgkin lymphoma. You are at increased risk if you have a weakened immune system or have certain types of infections.

Non-Hodgkin lymphoma can cause many symptoms, such as

• Swollen, painless lymph nodes in the neck, armpits or groin
• Unexplained weight loss
• Fever
• Soaking night sweats
• Coughing, trouble breathing or chest pain
• Weakness and tiredness that don’t go away
• Pain, swelling or a feeling of fullness in the abdomen

Your doctor will diagnose lymphoma with a physical exam, blood tests, a chest x-ray, and a biopsy. Treatments include chemotherapy, radiation therapy, targeted therapy, biological therapy, or therapy to remove proteins from the blood. Targeted therapy uses substances that attack cancer cells without harming normal cells. Biologic therapy boosts your body’s own ability to fight cancer. If you don’t have symptoms, you may not need treatment right away. This is called watchful waiting.

NIH: National Cancer Institute

• After chemotherapy – discharge (Medical Encyclopedia)
• B-cell leukemia/lymphoma panel (Medical Encyclopedia)
• Bone marrow transplant (Medical Encyclopedia)
• Burkitt lymphoma (Medical Encyclopedia)
• Lymph node biopsy (Medical Encyclopedia)
• Non-Hodgkin lymphoma (Medical Encyclopedia)
• Non-Hodgkin lymphoma – children (Medical Encyclopedia)
• Understanding Chemotherapy – NIH – Easy-to-Read (National Cancer Institute) – PDF
• What to Know about Brachytherapy (A Type of Internal Radiation Therapy) – NIH – Easy-to-Read (National Cancer Institute) – PDF
• What to Know about External Beam Radiation Therapy – NIH – Easy-to-Read (National Cancer Institute) – PDF

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. In Sézary syndrome, the cancerous T cells, called Sézary cells, are present in the blood, skin, and lymph nodes. A characteristic of Sézary cells is an abnormally shaped nucleus, described as cerebriform.People with Sézary syndrome develop a red, severely itchy rash (erythroderma) that covers large portions of their body. Sézary cells are found in the rash. However, the skin cells themselves are not cancerous; the skin problems result when Sézary cells move from the blood into the skin. People with Sézary syndrome also have enlarged lymph nodes (lymphadenopathy). Other common signs and symptoms of this condition include hair loss (alopecia), skin swelling (edema), thickened skin on the palms of the hands and soles of the feet (palmoplantar keratoderma), abnormalities of the fingernails and toenails, and lower eyelids that turn outward (ectropion). Some people with Sézary syndrome are less able to control their body temperature than people without the condition.The cancerous T cells can spread to other organs in the body, including the lymph nodes, liver, spleen, and bone marrow. In addition, affected individuals have an increased risk of developing another lymphoma or other type of cancer.Sézary syndrome most often occurs in adults over age 60 and usually progresses rapidly; historically, affected individuals survived an average of 2 to 4 years after development of the condition, although survival has improved with newer treatments.Although Sézary syndrome is sometimes referred to as a variant of another cutaneous T-cell lymphoma called mycosis fungoides, these two cancers are generally considered separate conditions.