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

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
B-cell lymphoma		0012191
Lymphadenopathy	Swollen lymph nodes	0002716
30%-79% of people have these symptoms
Abnormality of bone marrow cell morphology		0005561
Anorexia		0002039
Fatigue	Tired Tiredness [ more ]	0012378
Fever		0001945
Splenomegaly	Increased spleen size	0001744
Weight loss		0001824
5%-29% of people have these symptoms
Abnormality of the gastrointestinal tract		0011024

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Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 9/1/2018

Do you have updated information on this disease? We want to hear from you.

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.

Acalabrutinib (Brand name: Calquence) - Manufactured by Acerta Pharma BV
FDA-approved indication: Treatment of adult patients with mantle cell lymphoma (MCL) who have received at least one prior therapy.
National Library of Medicine Drug Information Portal
Medline Plus Health Information
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

Related Diseases Related Diseases

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 diagnoses include follicular lymphoma (see this term) and other forms of lymphoma. Visit the Orphanet disease page for more information.

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 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.

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.

Have a question? Contact a GARD Information Specialist.

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.