Rare Hematology News

Disease Profile

Diffuse Large B-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.
1-5 / 10 000

33,100 - 165,500

US Estimated

1-5 / 10 000

51,350 - 256,750

Europe Estimated

Age of onset

Adult

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ICD-10

C83.3

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)

DLBCL

Categories

Rare Cancers

Summary

Diffuse large Bcell lymphoma (DLBCL), a form of non-Hodgkin lymphoma, is the most common blood cancer. Lymphomas occur when cells of the immune system, known as B lymphocytes, grow and multiply uncontrollably. DLBCL occurs mostly in adults and is a fast-growing (aggressive) lymphoma. It can start in the lymph nodes or outside of the lymphatic system in the gastrointestinal tract, testes, thyroid, skin, breast, bone, or brain.[1] Often, the first sign of DLBCL is a painless rapid swelling in the neck, armpit, abdomen, or groin caused by enlarged lymph nodes. For some people, the swelling may be painful. Other symptoms include night sweats, unexplained fevers, and weight loss.[1][2]

Treatment may differ depending on the location of the tumor and the subtype of lymphoma.[1][3] For those who have advanced DCBCL and have not been treated previously, a combination of chemotherapy and the monoclonal antibody rituximab (Rituxan) (R-CHOP) may be tried.[1][2][3]. In addition, as of October 2017, axicabtagene ciloleucel (brand name: Yescarta), a type of gene therapy, has been approved by the United States FDA to treat DCBCL that is not responding to at least two treatment attempts or has returned after being treated before.[4] A stem cell transplant may also be an option if DLBCL returns or relapses. [1]

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.

  • Tisagenlecleucel-T(Brand name: Kymriah) Manufactured by Novartis Pharmaceuticals Corporation
    FDA-approved indication: May 2018, KYMRIAH is a CD19-directed genetically modified autologous Tcell immunotherapy indicated for the treatment of patients with diffuse large B-cell lymphoma, high-grade B-cell lymphoma, or DLBCL arising from follicular lymphoma who received two or more lines of systemic therapy.
    National Library of Medicine Drug Information Portal
    Medline Plus Health Information
  • Rituximab and hyaluronidase human(Brand name: Rituxan Hycela) Manufactured by Genentech, Inc.
    FDA-approved indication: Treatment of adult patients with previously untreated diffuse large B-cell lymphoma in combination with cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP) or other anthracycline-based chemotherapy regimens.
    National Library of Medicine Drug Information Portal
  • Axicabtagene ciloleucel(Brand name: Yescarta) Manufactured by Kite Pharma, Inc.
    FDA-approved indication: Treatment of adult patients with relapsed or refractory large B-cell lymphoma after two or more lines of systemic therapy, including diffuse large B-cell lymphoma (DLBCL) not otherwise specified, primary mediastinal large B-cell lymphoma, high-grade B-cell lymphoma, and DLBCL arising from follicular lymphoma.
    National Library of Medicine Drug Information Portal
    Medline Plus Health Information

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.

In-Depth Information

  • 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.

References

  1. Diffuse Large B-Cell Lymphoma. Lymphoma Research Foundation. December 2016; https://www.lymphoma.org/site/pp.asp?c=bkLTKaOQLmK8E&b=6300153.
  2. Freedman, Arnold and Friedberg, Jonathan. Patient information: Diffuse large B cell lymphoma in adults (Beyond the Basics). UpToDate. August 15 2017; https://www.uptodate.com/contents/diffuse-large-b-cell-lymphoma-in-adults-beyond-the-basics.
  3. Ghandi, S. Diffuse Large Cell Lymphoma. MedScape Reference. October 21, 2017; https://emedicine.medscape.com/article/202969-overview.
  4. FDA approves axicabtagene ciloleucel for large B-cell lymphoma. United States Food and Drug Administration (FDA). October 25, 2017; https://www.fda.gov/Drugs/InformationOnDrugs/ApprovedDrugs/ucm581296.htm.