Sézary syndrome

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at January 11, 2017
StartDiseasesSézary syndrome

Sézary syndrome is a type of cutaneous T-cell lymphoma which falls under the umbrella of non-Hodgkin's lymphoma. Cutaneous T-cell lymphomas are lymphoproliferative disorders. Sézary syndrome involves lymphocytes growing abnormally and cancerously. Lymphocytes are a type of white blood cell. Affecting the blood, Sézary syndrome causes a number of symptoms that manifest as a severe rash that covers the majority of the body. Synonyms for this condition include Sézary's erythroderma and Sézary's lymphoma.

Contents

Definition & Facts

Sézary syndrome is a type of lymphoma. It affects the T-cells which are lymphocytes that are produced in the bone marrow and recognize pathogens or invaders, precipitating an immune response. B-cells produce antibodies that attach to the antigens of the pathogens and fight infection.

B-cells lymphomas are more common than T-cell lymphomas. Whereas B-cell lymphomas comprise 85 percent of all non-Hodgkin's lymphomas, T-cell lymphomas comprise 10 to 15 percent of all non-Hodgkin's lymphomas.

Sézary syndrome typically afflicts patients at around age 50. It tends to afflict men more than women. It is considered a rare type of cancer, and no preventable risk factors are associated with the condition. There is still debate whether Sézary syndrome is a distinct disorder from mycosis fungoides or an advanced form of it.

Symptoms & Complaints

Symptoms of this condition include the presence of a rash that covers the majority of the body. These rashes are red, irritating, and itchy.

Rashes can go through phases. When beginning as mycosis fungoides, the rashes can look alternately like eczema, a hard lesion, and a tumor.

The lymph nodes may be swollen. Alopecia or hair loss is another symptom. Sézary syndrome is an advanced cancer, so by the time it is diagnosed, the cancerous lymphocytes have spread throughout the blood.

Causes

The causes of Sézary syndrome are unknown. It is not linked to any risk factors like smoking or obesity. Chromosomal abnormalities are present, implicating chromosome 10, chromosome 17, and chromosome 8. Family history does not appear to be a contributing factor in causing this condition, and it is not known to be inherited.

Diagnosis & Test

The diagnostic process will involve the health care professional taking the patient's medical history, family history, and a list of their symptoms. The patient's skin will closely examined and rashes will be noted. The physician will examine the skin to determine which kind of rash it is.

A battery of blood tests will be conducted. These include a complete blood count which will examine the amount of red blood cells, hemoglobin, and white blood cells. Blood tests will also seek to identify antigens to cancer. Flow cytometry is a test that examines the physical characteristics of the blood cells.

A biopsy may also be performed of the rashes or tumors to determine if they are cancerous. A biopsy may also be taken of the lymph nodes to assess whether or not the cancer has spread to them. The bone marrow may undergo biopsy as well. This biopsy will likely take the form of a fine-needle biopsy in which a needle is inserted into the bone marrow and a tissue sample is extracted.

Various imaging studies might be conducted to detect the presence of tumors and determine the state of metastasis. These could include computed tomography (CT) scans, positron emission tomography (PET) scans, magnetic resonance imaging (MRI) scans, ultrasounds, and X-rays, particularly chest X-rays. Staging of mycosis fungoides include the following:

  • Stage IA – under 10 percent of the body has a rash
  • Stage IB – 10 percent or more has a rash
  • Stage IIA – The rash exists; the cancer has not metastasized to lymph nodes, but they are swollen
  • Stage IIB – There is a tumor on the skin; the cancer has not metastasized to the lymph nodes, but they are swollen
  • Stage III – Most of the skin is irritated and has redness, the blood is affected, and there are tumors or plaques, but the cancer has not spread to the lymph nodes.
  • Stage IVA - Most of the skin is irritated and is covered in a rash and either the cancer has spread to the blood but not the lymph nodes or it hasn't spread to the blood but it has spread to the lymph nodes
  • Stage IVB – In addition to most of the skin being covered in a rash, cancer has spread to other organs. It may or may not have spread to the lymph nodes and blood.
  • Stage IV – Sézary syndrome – In addition to the rash covering 80 percent or more of the body, the cancer has definitely spread to the blood and possibly other organs and the lymph nodes.

Treatment & Therapy

Sézary syndrome is an advanced cancer and thus is difficult to treat. Biologic therapy, chemotherapy, and radiation therapy are all treatment approaches that are used to treat Sézary syndrome. Corticosteroids may be used to reduce inflammation and swelling. An example of a medication used during the course of biologic therapy includes interferon. Biologic therapy attempts to strengthen the body's immune system in order to fight the cancer.

Examples of chemotherapy drugs include gemcitabine, methotrexate, and liposomal doxorubicin. Pentostatin is another chemotherapy drug that may be used in treating this condition. Radiation therapy uses high-energy radiation to kill cancer cells. Clinical trials are also ongoing.

There are a number of additional therapies that may help abate symptoms as well as mitigate side effects of chemotherapy if not cure the disease. These include meditation, yoga, relaxation techniques, and acupuncture.

Prevention & Prophylaxis

There is no way to prevent this type of cancer because its causes remain unknown and it is not linked to any preventable lifestyle risks. Unlike other cancers linked to smoking, obesity, or sedentary lifestyle, health care researchers are unable to link Sézary syndrome to any particular risk factors.

Regular doctor's visits and physical examinations may help detect the condition before it has metastasized and become Sézary syndrome. Early diagnosis and early treatment enable better treatment outcomes overall.