Burkitt Lymphoma

  • Medical Author:
    Charles Patrick Davis, MD, PhD

    Dr. Charles "Pat" Davis, MD, PhD, is a board certified Emergency Medicine doctor who currently practices as a consultant and staff member for hospitals. He has a PhD in Microbiology (UT at Austin), and the MD (Univ. Texas Medical Branch, Galveston). He is a Clinical Professor (retired) in the Division of Emergency Medicine, UT Health Science Center at San Antonio, and has been the Chief of Emergency Medicine at UT Medical Branch and at UTHSCSA with over 250 publications.

  • Medical Editor: Melissa Conrad Stöppler, MD
    Melissa Conrad Stöppler, MD

    Melissa Conrad Stöppler, MD

    Melissa Conrad Stöppler, MD, is a U.S. board-certified Anatomic Pathologist with subspecialty training in the fields of Experimental and Molecular Pathology. Dr. Stöppler's educational background includes a BA with Highest Distinction from the University of Virginia and an MD from the University of North Carolina. She completed residency training in Anatomic Pathology at Georgetown University followed by subspecialty fellowship training in molecular diagnostics and experimental pathology.

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Burkitt lymphoma facts

  • Burkitt lymphomas are aggressive B-cell cancers that require urgent diagnosis and therapy.
  • The cause of these lymphomas is not known, but does involve a change in chromosome 8 and may be related to infections such as malaria and/or Epstein-Barr virus (EBV) infections.
  • There are many signs and symptoms associated with Burkitt's lymphoma; most commonly, the symptoms include abdominal and/or jaw masses, nausea and vomiting, headaches, visual impairment, and possibly renal, gastrointestinal, and other central nervous system problems.
  • Diagnosis is by tissue biopsy although other studies are usually done to further define and localize this cancer.
  • The two major staging systems for Burkitt lymphomas are from the National Cancer Institute and the Ann Arbor and St. Jude/Murphy systems.
  • The prognosis for children is good and for adults is fair if the diagnosis and treatment begin early.
  • Follow-up care is important to monitor possible complications and/or reoccurrence of the disease.

What are Burkitt lymphomas?

Burkitt lymphomas are aggressive B-cell cancers (non-Hodgkin type of lymphomas) that frequently involve aggressive progression in the bone marrow and in the central nervous system. Burkitt lymphoma (also known as small non-cleaved cell lymphoma and Burkitt's lymphoma) has a variant termed Burkitt-like lymphoma or BLL. Other types of Burkitt lymphoma are termed as follows:

  • Endemic or African is a type that usually involves the jaw and the facial bones.
  • Sporadic Burkitt lymphoma involves abdominal tumors.
  • Immunodeficiency-related Burkitt lymphoma has lymph node and bone marrow involvement.

The WHO (World Health Organization) suggests that there is little difference between Burkitt lymphoma and BLL and considers them essentially the same disease. The other variants are classified under the term Burkitt lymphoma. For the purposes of this article, we will discuss only Burkitt lymphoma and will not discuss small differences in subtypes. Because of their rapid progression, these lymphomas are considered medical emergencies that require fast diagnosis and treatment.

Burkitt lymphomas comprise about 1% to 2% of all lymphomas. The African variety usually starts as a jaw tumor while in United States, they usually begin as an abdominal tumor. About 90% of these lymphomas occur in males. In Africa, they occur mainly in children while in United States, the average age of onset is 30 years.

What causes Burkitt lymphoma?

The exact cause of Burkitt lymphoma is not known. However, the lymphomas are characterized by a translocation and deregulation of a gene (c-myc) on chromosome 8. In addition, there has been an association between Burkitt lymphoma and a high incidence of malaria and/or infection with Epstein-Barr virus (EBV). Additionally, patients with a depressed immune response have also been noted to have a higher incidence of Burkitt lymphoma.

What are the symptoms and signs of Burkitt lymphoma?

Signs and symptoms of Burkitt lymphoma are somewhat similar in all types and may include the following:

Abdominal symptoms are more prominent with Burkitt lymphoma seen in the US, while endemic (African) Burkitt lymphoma tends to cause more jaw-related symptoms.

How is Burkitt lymphoma diagnosed?

Diagnosis of Burkitt lymphoma depends upon pathologic evaluation of the involved tissue; consequently a bone marrow biopsy or aspiration is sometimes required and/or a tissue sample (for example, lymph node biopsy or a jaw tissue mass biopsy is done.). In addition to the biopsy, laboratory testing is usually done, and imaging studies such as CT scanning and echocardiography help determine the extent of the disease. Other procedures or studies that may be performed are removal of fluid collections in the abdomen or thorax for cytogenetic evaluation.

How is the staging of Burkitt lymphoma determined?

The stage of a tumor is the extent to which it has spread in the body. Unfortunately, different researchers have different ways of staging Burkitt lymphoma. The National Cancer Institute has a five stage system and is as follows:

  • A: Single solitary extra-abdominal site
  • AR: Intra-abdominal, more than 90% of tumor resected
  • B: Multiple extra-abdominal tumors
  • C: Intra-abdominal tumor
  • D: Intra-abdominal plus 1 or more extra-abdominal sites

The Michigan Ann Arbor system and St. Jude/Murphy system uses the following stages:

  • Stage I: Single tumor (extranodal or outside a lymph node) or single anatomic area
  • Stage II: Single tumor (extranodal) with regional node involved, primary gastrointestinal tumor and/or lymphoma involving nodal areas on the same side of the diaphragm
  • Stage IIR: Completely resected intra-abdominal disease
  • Stage III: Lymphoma involving sites on opposite sides of the diaphragm, all primary intrathoracic tumors, all paraspinal or epidural tumors, and/or extensive intra-abdominal disease

Patients should discuss which staging system their doctors use.

What is the treatment for Burkitt lymphoma?

Because Burkitt lymphomas are very aggressive and fast-growing cancers, people with the disease should be quickly evaluated by an oncologist and/or hematologist for aggressive chemotherapy. Because of the risk of tumor lysis syndrome (an emergency condition caused by lysis of tumor cells with the release of large amounts of tumor cell contents like nucleic acids, potassium, and uric acid into the body, resulting in renal, vascular, and inflammatory problems), patients will need close observation while undergoing therapy. Currently, there is no role for surgery or radiation therapy in treating these lymphoma types.

What is the prognosis for patients with Burkitt lymphoma?

The prognosis for about 90% of pediatric patients and up to 50% to 60% of adults is good, with long-term survival expected if the patient is quickly diagnosed and treated appropriately with chemotherapy. The prognosis for adults is more guarded (see survival rate section below).

What are the survival rates for patients with Burkitt lymphoma?

Adults usually do not do as well as children, especially if they have bone marrow involvement; cytogenetics of adult cells is more complex, resulting in aggressive disease. Survival for children can be as high as 90% as compared to about 50% to 60% for adults. For adults with low risk disease, the 5-year survival rate is about 71%, while high-risk aggressive lymphomas have about a 29% 5-year survival rate. The survival rate decreases as the patient's age increases.

What is the follow-up care for Burkitt lymphoma?

Patients who undergo chemotherapy may develop complications such as tumor lysis syndrome, renal failure, heart problems, infections, sterility, or other cancers. Consequently, patients need continual follow-up care to monitor for any complications that may develop, including recurrence of their lymphoma.

REFERENCES:

“Types of Non-Hodgkin Lymphoma.” American Cancer Society. 26 Aug 2014.

Kanbar, A. H., et al. “Burkitt Lymphoma and Burkitt-like Lymphoma.” Medscape. 3 Mar 2014.

Last Editorial Review: 11/20/2015

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Reviewed on 11/20/2015
References
REFERENCES:

“Types of Non-Hodgkin Lymphoma.” American Cancer Society. 26 Aug 2014.

Kanbar, A. H., et al. “Burkitt Lymphoma and Burkitt-like Lymphoma.” Medscape. 3 Mar 2014.

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