Rare Nephrology News

Disease Profile

Mantle cell lymphoma

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
1-9 / 100 000

3,310 - 29,790

US Estimated

1-9 / 100 000

5,135 - 46,215

Europe Estimated

Age of onset

Adult

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ICD-10

C83.1

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Lymphoma, mantle cell

Categories

Blood Diseases; Rare Cancers

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]

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]

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.

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

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]

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

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]

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.

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

    Organizations Providing General Support

      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

        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 Merck Manual for health care professionals provides information on Mantle cell lymphoma.
        • 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.

          References

          1. Mantle Cell Lymphoma. National Organization for Rare Disorders (NORD). 2005; https://rarediseases.org/rare-diseases/mantle-cell-lymphoma/.
          2. O’Connor OA and Vose JM. Mantle Cell Lymphoma: Getting the Facts. Lymphoma Research Foundation.. https://www.lymphoma.org/aboutlymphoma/nhl/mcl/. Accessed 7/11/2011.
          3. Non Hodgkin Lymphoma. American Cancer Society. October 2010; https://www.cancer.org/cancer/non-hodgkin-lymphoma.html. Accessed 7/11/2011.
          4. Mantle Cell Lymphomas. Lymphomation. June 2011; https://www.lymphomation.org/type-MCL.htm. Accessed 7/11/2011.
          5. Adult Non-Hodgkin Lymphoma Treatment–Patient Version (PDQ®). National Cancer Insitute. March 3, 2016; https://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq. Accessed 4/27/2016.
          6. Freedman AS, Friedberg JW. Initial treatment of mantle cell lymphoma. In: Lister A. UpToDate. Waltham, MA: UpToDate; November 13, 2015; https://www.uptodate.com/contents/initial-treatment-of-mantle-cell-lymphoma.

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