National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Sezary syndrome


Other Names:
Sezary's lymphoma; Sézary syndrome
Categories:
This disease is grouped under:
Sezary syndrome is an aggressive form of cutaneous T-cell lymphoma which is a group of disorders that occur when T-cells (a type of white blood cell) become cancerous and affect the skin. It is characterized by a widespread red rash that may cover most of the body, the presence of cancerous T cells (called Sezary cells) in the blood, and abnormally enlarged lymph nodes. Other signs and symptoms may include intense itchiness, scaling and peeling of the skin; fever; weight loss; hair loss; outward turning of the eyelids (ectropion); palmoplantar keratoderma; malformation of the nails; and hepatosplenomegaly. The exact cause of Sezary syndrome is currently unknown. Treatment varies based on the signs and symptoms present in each person and the severity of the condition.[1][2]
Last updated: 8/3/2015
Sezary syndrome is an aggressive form of cutaneous T-cell lymphoma which is a group of disorders that occur when T-cells (a type of white blood cell) become cancerous and affect the skin. In Sezary syndrome, specifically, the cancerous T cells are called Sezary cells and are found in the skin, lymph nodes, and blood. They can also spread to other organs in the body, including the liver, spleen, and bone marrow.[1][3]

Although Sezary syndrome can affect people of all ages, it is most commonly diagnosed in adults over age 60. The signs and symptoms of this condition can vary but may include:[1][3]
  • A red, itchy rash that covers large portions of the body
  • Enlarged lymph nodes
  • Alopecia (hair loss)
  • Thickened skin on the palms of the hands and soles of the feet
  • Abnormalities of the fingernails and toenails
  • Ectropion
  • Hepatosplenomegaly (enlarged liver and spleen)

Affected people may also have an increased risk of developing another lymphoma or other type of cancer.[3]
Last updated: 7/30/2015

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Abnormal lymphocyte morphology 0004332
Cutaneous T-cell lymphoma 0012192
Dry skin 0000958
Erythroderma 0001019
Lichenification 0100725
Lymphadenopathy
Swollen lymph nodes
0002716
Neoplasm of the skin
Skin tumors
Tumor of the skin
[ more ] 		0008069
Pruritus
Itching
Itchy skin
Skin itching
[ more ] 		0000989
30%-79% of people have these symptoms
Alopecia
Hair loss
0001596
Hepatomegaly
Enlarged liver
0002240
Immunodeficiency
Decreased immune function
0002721
Nail dystrophy
Poor nail formation
0008404
Palmoplantar keratoderma
Thickening of palms and soles
0000982
Splenomegaly
Increased spleen size
0001744
5%-29% of people have these symptoms
Abnormal facial shape
Unusual facial appearance
0001999
Abnormal immunoglobulin level 0010701
Abnormal pleura morphology 0002103
Ectropion
Eyelid turned out
0000656
Edema
Fluid retention
Water retention
[ more ] 		0000969
Gangrene
Death of body tissue due to lack of blood flow or infection
0100758
Irregular hyperpigmentation 0007400
Peripheral neuropathy 0009830
Skeletal muscle atrophy
Muscle degeneration
Muscle wasting
[ more ] 		0003202
Tremor 0001337
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Last updated: 7/1/2020
Although a small percentage of cases of Sezary syndrome are associated with human T-lymphotropic viruses type 1 and type 2, the underlying cause of most cases is currently unknown.[3] In people affected by the condition, the cancerous T-cells (also called Sezary cells) usually have one or more chromosomal abnormalities. These genetic changes are considered "somatic" because they are acquired during a person's lifetime, are not inherited and only affect the DNA of the cancerous cells. Although any chromosome can be affected, people with Sezary syndrome often have deletions of DNA from chromosomes 10 and 17 or duplications of DNA on chromosomes 8 and 17. However, it is unclear whether these alterations play a role in the development of the condition.[4]
Last updated: 7/30/2015
The underlying cause of Sezary syndrome is unknown in most cases. However, it generally occurs sporadically in people with no family history of the condition.[4]
Last updated: 7/30/2015
A diagnosis of Sezary syndrome is often suspected in people with characteristic signs and symptoms. Additional testing can then be ordered to confirm the diagnosis. This may include:[2][3]
  • A skin biopsy
  • A complete blood count
  • Peripheral blood smear
  • Immunophenotyping
  • T-cell receptor (TCR) gene rearrangement test
  • Flow cytometry

To learn more about the diagnosis of Sezary syndrome, including specific information about each of the tests listed above, please visit the National Cancer Institute's Web site. Simply click on the link and scroll down to the section entitled "tests that examine the skin and blood are used to detect (find) and diagnose mycosis fungoides and the Sezary syndrome"
Last updated: 7/30/2015
In general, there are six different treatment options available to people with Sezary syndrome. These include:[2]
  • Photodynamic therapy
  • Radiation therapy
  • Chemotherapy
  • Other drug therapy (i.e. topical steriods, retinoids)
  • Biologic therapy
  • Targeted therapy

To learn more about the treatment and management of Sezary syndrome, including specific information about each of the treatments listed above, please visit the National Cancer Institute's Web site. Simply click on the link to access this resource.
Last updated: 7/30/2015

FDA-Approved Treatments

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.

  • Mogamulizumab (Brand name: Poteligeo) - Manufactured by Kyowa Kirin Pharmaceutical Development Inc.
    FDA-approved indication: August 2018, mogamulizumab (Poteligeo) was approved for the treatment of adult patients with relapsed or refractory mycosis fungoides (MF) or Sézary syndrome (SS) after at least one prior systemic therapy.
    National Library of Medicine Drug Information Portal
The long-term outlook (prognosis) for people with Sezary syndrome is generally poor. Sezary syndrome is difficult to cure. Treatment is usually palliative, with the intention of relief of symptoms and improvement in the quality of life. Median survival for patients with Sezary syndrome has been reported to be 2 to 4 years after development of the condition, although survival has improved with newer treatments. The disease-specific 5-year survival rate has been reported to be 24%.[2][4]
Last updated: 8/3/2015

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
Differential diagnosis includes adverse drug reactions, classical mycosis fungoides and other forms of primary cutaneous T cell lymphoma (see these terms) as well as other causes of erythroderma such as psoriasis, atopic dermatitis and pityriasis rubra pilaris (see this term).
Visit the Orphanet disease page for more information.

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Sezary syndrome. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.
  • Orphanet lists European clinical trials, research studies, and patient registries enrolling people with this condition. 
  • The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. Although these projects may not conduct studies on humans, you may want to contact the investigators to learn more. To search for studies, enter the disease name in the "Text Search" box. Then click "Submit Query".

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Organizations Providing General Support

Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.

Financial Resources

  • Patient Access Network Foundation (PAN Foundation) has Assistance Programs for those with health insurance who reside in the United States. The disease fund status can change over time, so you may need to check back if funds are not currently available. 

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
  • Genetics Home Reference (GHR) contains information on Sezary syndrome. This website is maintained by the National Library of Medicine.
  • The National Cancer Institute provides the most current information on cancer for patients, health professionals, and the general public.
  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Sezary syndrome. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • Is Sezary syndrome hereditary? My father had this and now I think my sister has it. See answer

  • Can Sezary syndrome be cured? See answer


  1. Lauren C Pinter-Brown, MD. Cutaneous T-Cell Lymphoma. Medscape Reference. March 2015; http://emedicine.medscape.com/article/2139720-overview#showall.
  2. Mycosis Fungoides and the Sézary Syndrome Treatment (PDQ®). National Cancer Institute. May 2015; http://www.cancer.gov/types/lymphoma/patient/mycosis-fungoides-treatment-pdq.
  3. Elise A Olsen, MD; Alain H Rook, MD. UpToDate. Clinical presentation, pathologic features, and diagnosis of Sézary syndrome. May 2013; Accessed 7/30/2015.
  4. Sézary syndrome. Genetics Home Reference. March 2013; http://ghr.nlm.nih.gov/condition/sezary-syndrome.