Fast Facts: Sézary Syndrome
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The Cutaneous Lymphoma Foundation is an independent, non-profit patient advocacy
organization dedicated to supporting every person with cutaneous lymphoma by
promoting awareness and education, advancing patient care, and facilitating research.
Fast Facts: Sézary Syndrome
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, David Geffen School of Medicine and director,
Lymphoma Program, UCLA School of Medicine, Los Angeles, California.
What is Lymphoma? kin lymphoma comprise more than 67 subtypes of
Lymphoma is a cancer of the white blood cells, lymphoma. There are six primary types of Hodg-
namely lymphocytes, which constitute the lym- kin lymphoma and as many as 61 types of non-
phatic system. The two main types of lymphoma Hodgkin lymphoma.
are Hodgkin lymphoma and non-Hodgkin lym-
phoma (NHL). Lymphoma is the most common Nearly all non-Hodgkin lymphoma cases occur
blood cancer and the third most common cancer in adults. The average age of diagnosis is usually
of childhood. Lymphoma occurs when lympho- in the early- to mid-60s, but lymphomas can de-
cytes grow abnormally. velop at any age. While scientists do not know the
exact causes of non-Hodgkin lymphoma, they do
The body has two main types of lymphocytes: B know that it is not caused by injury or by coming
lymphocytes, or B-cells, and T lymphocytes, or T- in contact with someone with the disease. Most
cells. B-cells play an important role in making an- people diagnosed with NHL have no known
tibodies to fight bacterial infections, while T-cells risk factors, although increasingly many scientists
play a role in fighting viruses and organ rejection believe infections may contribute to the develop-
in transplant patients. Although both cell types ment of certain types of non-Hodgkin lymphoma.
can develop into lymphomas, B-cell lymphomas According to the American Cancer Society, there
are more common, comprising nearly 85 percent were approximately 66,120 new cases of NHL di-
of all non-Hodgkin lymphomas. Like normal agnosed in 2008. The disease is more common in
lymphocytes, those that become malignant can men than in women.
grow in any part of the body, including the lymph
nodes, spleen, bone marrow, blood or other or- What Is T-Cell Lymphoma?
gans, including the liver, lung and kidney. Lym- T-cell lymphoma is a disease in which T lympho-
phoma has been shown to grow in virtually every cyte cells become cancerous. These lymphomas
organ of the body. account for between ten percent and 15 percent of
all cases of non-Hodgkin lymphoma in the Unit-
What is Non-Hodgkin Lymphoma? ed States (approximately 5,000 to 6,000 cases) a
Together, Hodgkin lymphoma and non-Hodg- year, although some forms of T-cell lymphoma
The Cutaneous Lymphoma Foundation is a 501(c)(3) nonprofit organization supported by tax-deductible donationsare more common in Asia and in other parts of more common in men than women. There are no
the world. There are many different types of T- known risk factors for this type of cutaneous T-
cell lymphoma and some are extremely rare, oc- cell lymphoma.
curring in only a few patients per year through-
out the world. Most T-cell lymphomas can be How Sézary Syndrome Is Diagnosed
classified into two broad categories: aggressive and Staged
(fast-growing) or indolent (slow-growing). Many of the same procedures used to diag-
nose and stage other types of cutaneous T-
One of the most common forms of T-cell lym- cell lymphomas are used in Sézary syndrome,
phoma is cutaneous, or skin, lymphoma, because including a physical exam and history; blood
it starts in the lymphocytes in the skin. Cutane- tests to identify antigens, or markers, on the
ous lymphoma actually describes many different surface of the cells in the blood; a skin and/or
disorders with various signs and symptoms, out- lymph node biopsy (removal of a small piece of
comes and treatment considerations. tissue) for examination under the microscope
by a pathologist (a doctor who studies tissues
What Is Sézary Syndrome? and cells to identify diseases); and a series of
The two most common types of cutaneous T- imaging tests such as CT (computerized ax-
cell lymphomas (CTCL) are mycosis fungoides, ial tomography), MRI (magnetic resonance
which is often indolent, appears as reddish skin imaging) and/or PET (positron emission to-
patches and can progress over many years, and mography) scans to determine if the cancer
an advanced and leukemic form of mycosis has spread to lymph nodes or other organs. In
fungoides called Sézary syndrome. Sézary syn- addition to these diagnostic tests, occasion-
drome is distinguished from mycosis fungoides ally a bone marrow biopsy may be necessary to
by the presence of malignant lymphocytes in the verify complete staging.
blood and is characterized by extensive thin red,
itchy rashes covering over 80 percent of the body. Because mycosis fungoides and Sézary syn-
In some cases, thicker, red patches (or plaques) drome are such rare cancers, it is important to
and tumors may also appear. In addition, these confirm a diagnosis by a dermatopathologist
symptoms may be accompanied by changes in or a hematopathologist, a pathologist who is
the nails, hair or eyelids or the presence of en- an expert in diagnosing lymphomas.
larged lymph nodes. The following staging system is used to deter-
mine the extent of mycosis fungoides:
There are about 3,000 new cases of mycosis • Stage IA—Less than 10 percent of the
fungoides each year in the U.S. and approximate- skin is covered in red patches
ly 15 percent of those are diagnosed as Sézary and/or plaques.
syndrome. Although this type of NHL can af- • Stage IB—Ten percent or more of
fect people of any age, Sézary syndrome usually the skin surface is covered in patches
occurs in adults ages 50 and over and is slightly and/or plaques.• Stage IIA—Any amount of the skin There are several types of standard treatment for
surface is covered with patches and/or Sézary syndrome:
plaques; lymph nodes are enlarged, but • Biologic, or immunotherapy, therapy is a
the cancer has not spread to them. treatment used to stimulate a patient’s own
• Stage IIB—One or more tumors are immune system to fight the cancer
found on the skin; lymph nodes may • Chemotherapy, a drug given either
be enlarged, but the cancer has not orally or through an infusion in a vein, to
spread to them. stop the growth of rapidly dividing
• Stage III—Nearly all of the skin is cancer cells
reddened and may have patches, • Extracorporeal photopheresis (ECP), a
plaques or tumors; lymph nodes may procedure used to expose the blood to
be enlarged, but the cancer has not ultraviolet light
spread to them. • Histone deacetylase inhibitors, a class of
• Stage IVA—Most of the skin area is drugs that cause a chemical change that
reddened and there is involvement of stops tumor cells from dividing
the blood with malignant cells or any • Phototherapy, for example, the drug
amount of the skin surface is covered psoralen and ultraviolet-A light radiation
with patches, plagues or tumors; cancer (PUVA) directed to the skin or skin-
has spread to the lymph nodes and the directed ultraviolet-B (UVB) or narrow
lymph nodes may be enlarged. band ultraviolet-B (NBUVB)
• Stage IVB—Most of the skin is • Radiation therapy, which uses high-energy
reddened or any amount of the skin X-rays or other types of radiation to kill
surface is covered with patches, plaques cancer cells or keep them from growing
or tumors; cancer has spread to other • Retinoids, which are drugs related to
organs; and lymph nodes may be vitamin A and can slow certain types of
enlarged whether cancer has spread to cancer cells
them or not. Some specific drugs include:
• Alemtuzumab (Campath), a
How Sézary Syndrome Is Treated monoclonal antibody
There are many effective therapies available to • Bexarotene (Targretin), a retinoid
treat Sézary syndrome. Because the disease is • Denileukin diftitox (Ontak), a
chronic and systemic (affecting the entire body), monoclonal antibody
Sézary syndrome is usually not treated with skin- • Gemcitabine (Gemzar), an
directed therapies alone. The specific treatment antimetabolite chemotherapy
for individual patients is based on a variety of fac- • Interferon alfa or interleukin-2, immune
tors, including the patient’s general health and stimulants that bind to specific cell-
stage of the disease. surface receptors
• Liposomal doxorubicin (Doxil), a chemotherapy that binds to DNA Participating in Clinical Trials
• Methotrexate (Trexall), an antimetabolite Clinical trials are crucial in identifying effective
chemotherapy, which blocks the drugs, prognostic strategies and determining
metabolism of cells optimal doses for lymphoma patients. Because
• Vorinostat (Zolinza), a histone mycosis fungoides and Sézary syndrome are such
deacetylase inhibitor rare diseases, finding enough patients to enroll in
Some common combination therapies include: clinical trials is often difficult. If you are interest-
• Bexarotene (Targretin) and ed in participating in a clinical trial, talk to your
interferon alpha doctor about an appropriate trial for you. To
• Bexarotene and phototherapy learn more about clinical trials, visit the Cutane-
• ECP (extracorporeal photopheresis) ous Lymphoma Foundation at www.clfounda-
and bexarotene tion.org.
• ECP and interferon alpha
• ECP, interferon alpha and bexarotene Are Complimentary and Alternative
• Phototherapy and interferon alpha Therapies Safe and Effective?
Some second-line chemotherapies for relapsed Complementary and alternative medicines are
(the recurrence of disease) or refractory (disease nonstandard therapies that may help patients
that is resistant to treatment) patients include: cope with their cancer and its treatment, but
• Bexarotene (Targretin) and denileukin that should not be used in place of standard
difitox (Ontak) treatment. No alternative therapy has ever been
• Chlorambucil (Leukeran) proven effective against lymphoma. However,
• Cyclophosphamide (Cytoxan) complementary therapies such as meditation,
• Pentostatin (Nipent) yoga, acupuncture, exercise, diet and relaxation
techniques have been shown to be effective in
Treatments Under Investigation combating some treatment side effects. Before
There are several single agent therapies and drug embarking on any complementary therapies,
combination regimens being studied in clinical patients should discuss the matter with their
trials in the treatment of mycosis fungoides and healthcare team. Certain unproven treatments,
Sézary syndrome. Some of those treatments in- including some herbal supplements, can inter-
clude: fere with standard lymphoma treatments or may
• Bortezomib (Velcade) cause serious side effects.
• Enzastaurin
• PDX (Pralatrexate) How to Prepare for
• PUVA therapy, plus interferon alfa Follow-Up Appointments
It is important for patients both during and after
treatment to be proactive in their healthcare, in-
cluding keeping a master file of medical records,
asking questions, reporting new symptoms,exercising and eating a balanced diet. In addi- these resources:
tion, patients who smoke should strongly con- Call: 248-644-9014
sider stopping. Follow-up visits for people with Website: www.clfoundation.org
Sezary syndrome often depends on the stage of The Lymphoma Research Foundation offers a
the disease and treatment and can range from as wide range of resources on the latest treatment
frequently as every few weeks when starting new and research advances, and services for coping
therapies that require monitoring to as little as with lymphoma. For more information about any
every six months. of these resources:
Typically, follow-up visits include physical ex- Call: 800-500-9976
aminations, blood tests and occasionally imaging E-mail: helpline@lymphoma.org
tests such as CT or PET scans. Besides deter- Website: www.lymphoma.org
mining disease recurrence, follow-up care can
help identify and resolve unusual side effects of How to Support Us
treatment. Your contribution helps promote awareness and
education about cutaneous lymphomas and sup-
Finding Support ports research. When you send a donation of any
Getting a lymphoma diagnosis can be frightening amount to the Cutaneous Lymphoma Founda-
and treatment can cause physical and emotional tion, you will receive a subscription to the Cuta-
discomfort. Connecting with other people who neous Lymphoma Foundation Forum, our quar-
have Sézary syndrome can be extremely helpful. terly newsletter.
The Cutaneous Lymphoma Foundation offers
information on patient assistance programs, free To make a donation, send a check or money
e-mail listservs, where to find clinical trials and order to:
the latest research on its website at www.clfoun- Cutaneous Lymphoma Foundation
dation.org. Support groups and online message P.O. Box 374
boards are often useful. One-to-one peer support Birmingham, MI 48102-0374
programs, such as the Lymphoma Research
Foundation’s Lymphoma Support Network at You may also donate online at
www.lymphoma.org, matches lymphoma sur- www.clfoundation.org
vivors (or caregivers) with volunteers who have
gone through similar experiences. Who We Are
The Cutaneous Lymphoma Foundation is an
Staying Informed independent, nonprofit patient advocacy orga-
The Cutaneous Lymphoma Foundation is a nization dedicated to supporting patients with
patient advocacy organization dedicated to edu- cutaneous lymphomas by promoting awareness
cating patients and caregivers about cutaneous and education, advancing patient care and facili-
lymphomas and improving access to safe and ef- tating research.
fective treatments. For more information aboutGlossary of Terms to identify areas in the body that are affected by
lymphoma. This test evaluates metabolic activity
Biopsy in different parts of the body using a radioisotope.
Removal of a small piece of tissue (for example, a
lymph node) for evaluation under a microscope. Relapse
The return of cancer after treatment. Lymphoma
Chemotherapy may recur in the area where it first started or it may
Treatment with “chemo” drugs to stop the growth relapse in another area of the skin.
of rapidly dividing cancer cells, including lympho-
ma cells. Refractory disease
A cancer that is resistant tor treatment.
Chemotherapy regimen
Combinations of anti-cancer drugs given at a Stage
certain dose in a specific sequence according to a The extent of cancer on the skin or in the body,
strict schedule. including whether the disease has spread from the
original site to other body parts.
CT or CAT (computerized axial tomogra-
phy) scan
This imaging test provides a series of detailed pic-
tures of the inside of the body using an X-ray ma-
chine linked to a computer.
Electron beam radiation
Contact Us
Radiation of the skin that does not penetrate to
internal organs. For more information about the Cutaneous
Lymphoma Foundation, please contact:
Lymph nodes Cutaneous Lymphoma Foundation
Small bean-shaped glands located in the small PO Box 374
vessels of the lymphatic system. Thousands are lo- Birmingham, MI 48012
cated throughout the body and are most easily felt 248-644-9014
in the neck, armpits and groin.
Web site: www.clfoundation.org
Lymphatic system
The vessels, tissues and organs that store and E-mail: Info@clfoundation.org
carry lymphocytes that fight infection and other
©2008 Cutaneous Lymphoma Foundation
diseases.
Because each person’s body and response to treatment
Lymphocyte is different, no individual should indulge in self-diag-
A type of white blood cell. nosis or embark upon any course of medical treatment
without first consulting with his or her physician.
PET (positron emission tomography) scan) Last Updated March 2008
PET scans may be used instead of gallium scans
This project was supported through the Lymphoma Research Foundation’s cooperative agreement (number IU58DP001110-01)
with the Centers for Disease Control and Prevention. Its contents are solely the responsibility of the authors and do not neces-
sarily represent the official views of the Centers for Disease Control and Prevention.You can also read
