What Is Sézary Syndrome?
The two most common types of cutaneous T-cell lymphomas (CTCL) are mycosis fungoides, which is often indolent, appears as reddish skin patches and can progress over many years, and an advanced and leukemic form of mycosis fungoides called Sézary syndrome. Sézary syndrome is distinguished from mycosis fungoides by the presence of malignant lymphocytes in the blood and is characterized by extensive thin red, itchy rashes covering over 80 percent of the body. In some cases, thicker, red patches (or plaques) and tumors may also appear. In addition, these symptoms may be accompanied by changes in the nails, hair or eyelids or the presence of enlarged lymph nodes.
There are about 3,000 new cases of mycosis fungoides each year in the U.S. and approximately 15 percent of those are diagnosed as Sézary syndrome. Although this type of NHL can affect people of any age, Sézary syndrome usually occurs in adults ages 50 and over and is slightly more common in men than women. There are no known risk factors for this type of cutaneous T-cell lymphoma.
How Sézary Syndrome Is Diagnosed and Staged?
Many of the same procedures used to diagnose and stage other types of cutaneous T-cell lymphomas are used in Sézary syndrome, including a physical exam and history; blood tests to identify antigens, or markers, on the surface of the cells in the blood; a skin and/or lymph node biopsy (removal of a small piece of tissue) for examination under the microscope by a pathologist (a doctor who studies tissues and cells to identify diseases); and a series of imaging tests such as CT (computerized axial tomography), MRI (magnetic resonance imaging) and/or PET (positron emission tomography) scans to determine if the cancer has spread to lymph nodes or other organs. In addition to these diagnostic tests, occasionally a bone marrow biopsy may be necessary to verify complete staging.
Because mycosis fungoides and Sézary syndrome are such rare cancers, it is important to confirm a diagnosis by a dermatopathologist or a hematopathologist, a pathologist who is an expert in diagnosing lymphomas.
The following staging system is used to determine the extent of mycosis fungoides:
How Sézary Syndrome Is Treated?
There are many effective therapies available to treat Sézary syndrome. Because the disease is chronic and systemic (affecting the entire body), Sézary syndrome is usually not treated with skin-directed therapies alone. The specific treatment for individual patients is based on a variety of factors, including the patient’s general health and stage of the disease.
There are several types of standard treatment for Sézary syndrome:
Some specific drugs include:
Some common combination therapies include:
Some second-line chemotherapies for relapsed (the recurrence of disease) or refractory (disease that is resistant to treatment) patients include:
Treatments Under Investigation
There are several single agent therapies and drug combination regimens being studied in clinical trials in the treatment of mycosis fungoides and Sézary syndrome. Some of those treatments include:
Participating in Clinical Trials
Clinical trials are crucial in identifying effective drugs, prognostic strategies and determining optimal doses for lymphoma patients. Because mycosis fungoides and Sézary syndrome are such rare diseases, finding enough patients to enroll in clinical trials is often difficult. If you are interested in participating in a clinical trial, talk to your doctor about an appropriate trial for you.
Are Complementary and Alternative Therapies Safe and Effective?
Complementary and alternative medicines are nonstandard therapies that may help patients cope with their cancer and its treatment, but that should not be used in place of standard treatment. No alternative therapy has ever been proven effective against lymphoma. However, complementary therapies such as meditation, yoga, acupuncture, exercise, diet and relaxation techniques have been shown to be effective in combating some treatment side effects. Before embarking on any complementary therapies, patients should discuss the matter with their healthcare team. Certain unproven treatments, including some herbal supplements, can interfere with standard lymphoma treatments or may cause serious side effects.
How to Prepare for Follow-Up Appointments
It is important for patients both during and after treatment to be proactive in their healthcare, including keeping a master file of medical records, asking questions, reporting new symptoms, exercising and eating a balanced diet. In addition, patients who smoke should strongly consider stopping. Follow-up visits for people with Sézary syndrome often depends on the stage of the disease and treatment and can range from as frequently as every few weeks when starting new therapies that require monitoring to as little as every six months.
Typically, follow-up visits include physical examinations, blood tests and occasionally imaging tests such as CT or PET scans. Besides determining disease recurrence, follow-up care can help identify and resolve unusual side effects of treatment
Expert review by:
Steven M. Horwitz, M.D., Assistant Attending, Lymphoma Service, Memorial Sloan-Kettering Cancer Center, New York City, New York;
Lauren Pinter-Brown, M.D., Clinical Professor of Medicine,UCLA School of Medicine, Los Angeles, California;
David Geffen School of Medicine and director, Lymphoma Program, UCLA School of Medicine, Los Angeles, California.