Mycosis fungoides

Title

Other Names:

Granuloma fungoides; Alibert-Bazin syndrome

Categories:

Rare Cancers

This disease is grouped under:

Cutaneous T-cell lymphoma

Summary Summary

Mycosis fungoides is a disease in which T-cell lymphocytes (a type of white blood cell) become malignant (cancerous) and affect the skin. This condition is one of the most common types of T-cell lymphoma. Mycosis fungoides is characterized by a scaly, red rash that develops on the skin, particularly on areas that are not usually exposed to the sun. The rash may last for months or years without causing any symptoms. Over time, a thin, reddened, eczema-like rash may develop, followed by thickened, red patches of skin. Finally, tumors form which may develop into ulcers and become infected. Mycosis fungoides is difficult to cure. Treatment is usually palliative, with the intention of relieving symptoms and improving the quality of life.^[1]

Last updated: 1/26/2011

Symptoms Symptoms

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
Percent of people who have these symptoms is not available through HPO
Eczema		0000964
Erythema		0010783
Lymphadenopathy	Swollen lymph nodes	0002716
Lymphoma	Cancer of lymphatic system	0002665
Neoplasm of the skin	Skin tumors Tumor of the skin [ more ]	0008069
Pruritus	Itching Itchy skin Skin itching [ more ]	0000989
Psoriasiform dermatitis		0003765
Skin plaque		0200035

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Last updated: 11/1/2018

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Treatment Treatment

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.

Brentuximub vedotin (Brand name: Adcetris) - Manufactured by Seattle Genetics, Inc
FDA-approved indication: Treatment for adult patients with CD30-expressing mycosis fungoides who have received prior systemic therapy.
National Library of Medicine Drug Information Portal
Medline Plus Health Information
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
Mechlorethamine (Brand name: Valchlor) - Manufactured by Ceptaris Therapeutics, Inc
FDA-approved indication: Topical treatment of Stage 1A and 1B mycosis fungoides-type cutaneous T-cell lymphoma in patients who have received prior skin-directed therapy.
National Library of Medicine Drug Information Portal
Medline Plus Health Information

Prognosis Prognosis

Mycosis fungoides (MF) has an indolent (low-grade) clinical course, which means that it may persist in one stage, or may slowly progress to another stage (from patches to thicker plaques and eventually to tumors) over years or sometimes decades.^[2] The condition is incurable in most affected individuals, with the exception of those with stage IA disease. The prognosis for each individual varies and is related to the disease stage at the time of diagnosis, the type and extent of skin lesions, and whether the disease is present in other areas of the body (extracutaneous).^[3]
Studies regarding the survival outcomes and prognosis have generally found that:

individuals with stage IA disease at the time od diagnosis who undergo treatment have a normal life expectancy (e.g. that expected based on age, sex and race) -- a 10-year survival rate of 97-98%.
individuals with stage IIB disease with cutaneous tumors have a median survival rate of 3.2 years (a 10-year survival rate of 42%).
those with stage III disease (generalized erythroderma) have a median survival rate of 4-6 years (a 10-year survival rate of 83%).
individuals with extracutaneous stage IVA (lymph nodes) or stage IVB (viscera) disease have a survival rate of less than 1.5 years (a 10-year survival rate of 20% for those with histologically documented lymph node involvement).
individuals with effaced lymph nodes, visceral involvement, and transformation to large T-cell lymphoma have an aggressive clinical course and usually die of systemic involvement or infections.

Other factors that may be associated with reduced survival and increased risk of disease progression include increased age, male sex, and increased lactate dehydrogenase (LDH).^[3]

Last updated: 3/7/2013

Find a Specialist Find a Specialist

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

To find a medical professional who specializes in genetics, you can ask your doctor for a referral or you can search for one yourself. Online directories are provided by the American College of Medical Genetics and the National Society of Genetic Counselors. If you need additional help, contact a GARD Information Specialist. You can also learn more about genetic consultations from Genetics Home Reference.

Research Research

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 Mycosis fungoides. 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.

Organizations Organizations

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

Cutaneous Lymphoma Foundation
PO Box 374
Birmingham, MI 48012
Telephone: 248-644-9014
Fax: 248-644-9014
E-mail: info@clfoundation.org
Website: http://www.clfoundation.org/

Do you know of an organization? We want to hear from you.

Living With Living With

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.

Learn More Learn More

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 Mycosis fungoides. 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.
MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
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.
Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
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 Mycosis fungoides. Click on the link to view a sample search on this topic.

News & Events News & Events

News

The NIH Undiagnosed Diseases Network Expands
September 26, 2018

GARD Answers GARD Answers

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

I have been recently diagnosed with mycosis fungoides. I haven't been staged yet. What can I expect? See answer

Have a question? Contact a GARD Information Specialist.

References References

Mycosis Fungoides and the Sézary Syndrome Treatment (PDQ®) . National Cancer Institute (NCI). 2010; http://www.cancer.gov/cancertopics/pdq/treatment/mycosisfungoides/Patient. Accessed 1/26/2011.
Cutaneous T-cell lymphoma. DermNet NZ. November 23, 2012; http://www.dermnet.org.nz/dermal-infiltrative/cutaneous-t-cell-lymphoma.html. Accessed 3/7/2013.
Lauren C Pinter-Brown. Cutaneous T-Cell Lymphoma. Medscape Reference. February 1, 2013; http://emedicine.medscape.com/article/2139720-overview. Accessed 3/7/2013.

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