Rare Dermatology News

Disease Profile

Burkitt 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.

Unknown

Age of onset

#N/A

ICD-10

#N/A

Inheritance

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

no.svg

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

no.svg

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.

no.svg

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

no.svg

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.

no.svg

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.

no.svg

Not applicable

no.svg

Other names (AKA)

Burkitt's lymphoma; BL

Categories

Blood Diseases; Rare Cancers

Summary

Burkitt lymphoma (BL) is a very fast-growing type of cancer. It is a form of Bcell non-Hodgkin's lymphoma.[1] There are 3 recognized forms of BL:

  • Endemic (African) the most common form, found mainly in central Africa, where it is associated with the Epstein Barr virus (EBV).[2] It is most common in children. This form often manifests as enlargement of the jaw or facial bones.[3]
  • Sporadic a rarer form, seen in all parts of the world, that often develops in the abdomen with bone marrow involvement. The kidneys, ovaries, breasts or other organs may also be involved.[3][2] This form commonly affects children and young adults.[4]
  • Immunodeficiency-associated occurs primarily in people with HIV infection, and less commonly in people with other immunodeficiency disorders or recipients of organ transplants.[2][5]

Signs and symptoms may differ depending on the form of BL and the organs or body systems involved.[2] When it spreads, weakness and fatigue often develop. Lymphoma cells may build up in the lymph nodes and other organs, causing swelling.[1] Central nervous system involvement is possible with all forms of BL, particularly when there is advanced-stage disease.[4]

The exact cause of BL is not known. EBV infection appears to play a role in virtually all cases of endemic (African) BL, and a minority of sporadic and immunodeficiency-associated BL.[5] While acquired (not inherited) genetic changes involving the MYC gene and other genes are present within BL cancer cells, it is unclear what causes these genetic changes to occur.[2][3]

Without timely treatment, BL is rapidly fatal. Treatment involves intensive chemotherapy, which includes chemotherapy to the fluid surrounding the brain and spinal cord.[1] The majority of people treated with aggressive therapy achieve long-term remission.[5]

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.

Medical Terms Other Names
Learn More:
HPO ID
30%-79% of people have these symptoms
Abnormality of bone marrow cell morphology
0005561
Hyperuricemia
High blood uric acid level
0002149
Increased lactate dehydrogenase level
0025435
Neoplasm of the oral cavity
0100649
5%-29% of people have these symptoms
Abdominal pain
Pain in stomach
Stomach pain

[ more ]

0002027
Abnormality of the liver
Abnormal liver
Liver abnormality

[ more ]

0001392
Abnormality of the lymph nodes
Abnormal lymph node histology
0002733
Abnormality of the ovary
Abnormality of the ovaries
0000137
Abnormality of the pancreas
0001732
Abnormality of the spleen
0001743
Decreased proportion of CD4-positive helper T cells
0005407
Gastrointestinal hemorrhage
Gastrointestinal bleeding
0002239
Intestinal obstruction
Bowel obstruction
Intestinal blockage

[ more ]

0005214
Nausea and vomiting
0002017
Percent of people who have these symptoms is not available through HPO
Burkitt lymphoma
0030080
Immunodeficiency
Decreased immune function
0002721
Somatic mutation
0001428
Sporadic
No previous family history
0003745

Diagnosis

Making a diagnosis for a genetic or rare disease can often be challenging. Healthcare professionals typically look at a person’s medical history, symptoms, physical exam, and laboratory test results in order to make a diagnosis. The following resources provide information relating to diagnosis and testing for this condition. If you have questions about getting a diagnosis, you should contact a healthcare professional.

Testing Resources

  • The Genetic Testing Registry (GTR) provides information about the genetic tests for this condition. The intended audience for the GTR is health care providers and researchers. Patients and consumers with specific questions about a genetic test should contact a health care provider or a genetics professional.

    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

      Social Networking Websites

        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

          • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
          • 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.

            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 Burkitt 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.
            • 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 Burkitt lymphoma. Click on the link to view a sample search on this topic.

              References

              1. Portlock CS. Burkitt Lymphoma. Merck Manual Consumer Version. https://www.merckmanuals.com/home/blood-disorders/lymphomas/burkitt-lymphoma. Accessed 9/25/2017.
              2. Kanbar AH. Burkitt Lymphoma and Burkitt-like Lymphoma. Medscape Reference. April 8, 2016; https://emedicine.medscape.com/article/1447602-overview.
              3. Portlock CS. Burkitt Lymphoma. Merck Manual Professional Version. October, 2012; https://www.merckmanuals.com/professional/hematology-and-oncology/lymphomas/burkitt-lymphoma.
              4. Dunleavy K, Little RF, Wilson WH. Update on Burkitt Lymphoma. Hematol Oncol Clin North Am. December, 2016; 30(6):1333-1343. https://www.ncbi.nlm.nih.gov/pubmed/27888884.
              5. Freedman AS, Aster JC. Epidemiology, clinical manifestations, pathologic features, and diagnosis of Burkitt lymphoma. UpToDate. Waltham, MA: UpToDate; August, 2017; https://www.uptodate.com/contents/epidemiology-clinical-manifestations-pathologic-features-and-diagnosis-of-burkitt-lymphoma.
              6. Hamosh A. Burkitt lymphoma; BL. OMIM. July 6, 2017; https://omim.org/entry/113970.
              7. Freedman AS, Friedberg JW. Treatment of Burkitt leukemia/lymphoma in adults. UpToDate. Waltham, MA: UpToDate; August, 2017; https://www.uptodate.com/contents/treatment-of-burkitt-leukemia-lymphoma-in-adults.
              8. Casulo C, Friedberg J. Treating Burkitt Lymphoma in Adults. Curr Hematol Malig Rep. September, 2015; 10(3):266-271. https://link.springer.com/article/10.1007/s11899-015-0263-4.
              9. Burkitt lymphoma. MedlinePlus. May 20, 2016; https://medlineplus.gov/ency/article/001308.htm.

              Rare Dermatology News