Mantle cell lymphoma

Title

Other Names:

Lymphoma, mantle cell

Categories:

Blood Diseases; Rare Cancers

Summary Summary

Mantle cell lymphoma (MCL) belongs to a group of diseases known as non-Hodgkin’s lymphomas (NHL). NHL's are cancers that affect the the lymphatic system (part of the immune system). In MCL, there are cancerous B-cells (a type of immune system cell). The cancerous B-cells are within a region of the lymph node known as the mantle zone.^[1] Although MCLs are slow-growing cancers, the cancer is usually widespread by the time it is diagnosed. In these situations, treatment must be intensive since MCL can become life threatening within a short period of time.^[2]^[3] MCL accounts for 6% of all NHL's and is mostly found in males during their early 60s.^[4]^[2]^[3]

Last updated: 4/27/2016

Symptoms Symptoms

Signs and symptoms of mantle cell lymphoma (MCL) may include swelling of the lymph nodes, fever, night sweats, weight loss, and fatigue. Some people have no to few signs of MCL leading to delayed diagnosis and treatment.^[2]^[5]

Of note, people with MCL may be at an increased risk for gastrointestinal issues, such as obstruction, intussusception, and multiple intestinal polyps, as well as a very high white blood cell count.^[6]

Last updated: 4/27/2016

The Human Phenotype Ontology (HPO) provides the following list of features that have been reported in people with this condition. Much of the information in the HPO comes from Orphanet, a European rare disease database. If available, the list includes a rough estimate of how common a feature is (its frequency). Frequencies are based on a specific study and may not be representative of all studies. You can use the MedlinePlus Medical Dictionary for definitions of the terms below.

Signs and Symptoms	Approximate number of patients (when available)
B-cell lymphoma	Very frequent (present in 80%-99% of cases)
Lymphadenopathy	Very frequent (present in 80%-99% of cases)
Abnormality of bone marrow cell morphology	Frequent (present in 30%-79% of cases)
Anorexia	Frequent (present in 30%-79% of cases)
Fatigue	Frequent (present in 30%-79% of cases)
Fever	Frequent (present in 30%-79% of cases)
Splenomegaly	Frequent (present in 30%-79% of cases)
Weight loss	Frequent (present in 30%-79% of cases)
Abnormality of the gastrointestinal tract	Occasional (present in 5%-29% of cases)

Last updated: 9/1/2017

Cause Cause

Most lymphomas are not inherited, but rather are acquired when the DNA within select body cells has been damaged (somatic DNA damage). Some risk factors for non-Hodgkin lymphomas (NHL) include age (older), gender (male), race (white), and having a condition which weakens the immune system, such as autoimmune conditions, certain genetic disorders, being on immune suppressing medications, HIV/AIDS, HTLV-1, Ebstein-Barr virus, and helicobacter pylori infection.^[5]

Last updated: 4/27/2016

Diagnosis Diagnosis

The following tests and procedures may be used to diagnose mantle cell lymphoma:^[5]

Physical exam and medical history
Flow cytometry
Bone marrow aspiration or biopsy
Lymph node biopsy (surgical removal of all or part of a lymph node)

If cancer is found, the following tests may be done to study the cancer cells:

Immunohistochemistry
Cytogenetic analysis
FISH
Immunophenotyping

Last updated: 4/27/2016

Treatment Treatment

Treatment of Mantle cell lymphoma (MCL) most often involves a combination of chemotherapy and immunotherapy. Additionally, hematopoietic stem cell transplantation may be considered in some cases. Surgery is usually not helpful.^[6]

Last updated: 4/27/2016

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.

Lenolidomide (Brand name: Revlimid)
FDA-approved indication: Treatment of mantle cell lymphoma whose disease has relapsed or progressed after two prior therapies, one of which included bortezomib.
National Library of Medicine Drug Information Portal
Bortezomib (Brand name: Velcade®) - Manufactured by Millennium Pharmaceuticals, Inc.
FDA-approved indication: Treatment of multiple myeloma patients who have received at least one prior therapy. Treatment of patients with mantle cell lymphoma.
National Library of Medicine Drug Information Portal
Medline Plus Health Information

Do you have updated information on this condition? Let us know.

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 studying or have studied Mantle cell lymphoma. 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.

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.
The Social Security Administration has included this condition in their Compassionate Allowances Initiative. This initiative speeds up the processing of disability claims for applicants with certain medical conditions that cause severe disability. More information about Compassionate Allowances and applying for Social Security disability is available online.

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

The Merck Manuals Online Medical Library provides information on this condition for patients and caregivers.
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 Mantle cell lymphoma. Click on the link to view a sample search on this topic.

News & Events News & Events

News

Don’t Miss the NCATS Toolkit for Patient-Focused Therapy Development Event
August 31, 2017
Funding Opportunities Now Available for the Undiagnosed Diseases Network (UDN) Phase II
August 30, 2017
IRDiRC Goals 2017-2027: New Rare Disease Research Goals for the Next Decade
August 10, 2017

NCATS Co-Sponsored Conferences

Advancing Symptom Clusters Research on Rare Cancers Wednesday, June 17, 2015
Location: NIH Neuroscience Building, Bethesda, MD
Description:
It is intended that the in-depth, interdisciplinary dialogue of this expert working group will formulate an emerging consensus on a working definition of symptom clusters and, specific gaps and opportunities that provide a foundation for a transformative strategic blueprint to guide future symptom cluster research in rare cancers. In addition, it is anticipated that workshop proceedings will inform a funding opportunity announcement.

Contact: Sue Marden, Ph.D.,(301) 496-9623,mardens@mail.nih.gov

Co-funding Institute(s): National Institute of Nursing Research, Office of Rare Diseases Research
Rare Tumors Initiative Symposium Strategies to Develop Therapies for Rare Tumors: Small Numbers, but Big Opportunities Wednesday, June 17, 2015
Location: NIH Natcher Conference Center, Bethesda, MD
Description: The goals of this symposium are to increase communication and build networks between researchers working in rare tumors across NIH, and to get input from patient groups, industry, and the FDA on how to overcome the biggest hurdles in the development of therapies for rare tumors. The Rare Tumors Initiative at NCI, CCR will use this information to prioritize goals for the future and will prepare a white paper summary to help guide researchers worldwide.

Contact: Dr. Karlyne Reilly,(301) 846-7518, reillyk@mail.nih.gov

Co-funding Institute(s): National Cancer Institute, Office of Rare Diseases Research
Overcoming Barriers to International Clinical Trials for Rare Cancers Friday, December 10, 2010
Location: Bethesda, Maryland
Description: The goals of this conference were to introduce key institutional players to the topic of international clinical trials in rare cancers and to establish an ongoing dialogue. Participants left the meeting with a set of specific priorities that need to be enacted to promote these trials. The meeting promoted consensus on the way that resources are prioritized to address rare cancers. Participants were asked to convey the content of the meeting to their constituencies and to follow up with pilot concepts.

Contact: Jack Welch, M.D., Ph.D., jack.welch@nih.govjack.welch@nih.gov

Co-funding Institute(s): National Cancer Institute, Office of Rare Diseases Research
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References References

Mantle Cell Lymphoma. National Organization for Rare Disorders (NORD). February 2011; http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Mantle%20Cell%20Lymphoma. Accessed 7/11/2011.
O’Connor OA and Vose JM. Mantle Cell Lymphoma: Getting the Facts. Lymphoma Research Foundation.. http://www.lymphoma.org/atf/cf/%7B0363CDD6-51B5-427B-BE48-E6AF871ACEC9%7D/MANTLE%20CELL.PDF. Accessed 7/11/2011.
Non Hodgkin Lymphoma. American Cancer Society. October 2010; http://www.cancer.org/acs/groups/cid/documents/webcontent/003126-pdf.pdf. Accessed 7/11/2011.
Mantle Cell Lymphomas. Lymphomation. June 2011; http://www.lymphomation.org/type-MCL.htm. Accessed 7/11/2011.
Adult Non-Hodgkin Lymphoma Treatment–Patient Version (PDQ®). National Cancer Insitute. March 3, 2016; http://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq. Accessed 4/27/2016.
Freedman AS, Friedberg JW. Initial treatment of mantle cell lymphoma. In: Lister A. UpToDate. Waltham, MA: UpToDate; November 13, 2015; Accessed 4/27/2016.