Burkitt Lymphoma

There are multiple types of Burkitt lymphoma. Burkitt lymphomas are types of non-Hodgkin's lymphoma that affect the bone marrow and central nervous system. Symptoms of Burkitt lymphoma may include nausea, vomiting, headache, fatigue, enlarged lymph nodes, and many other symptoms.

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%-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.

• 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). People with a depressed immune response have also been noted to have a higher incidence of Burkitt lymphoma.

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

• nausea and vomiting
• loss of appetite
• abdominal pain
• abdominal masses
• masses in the jaw
• renal failure
• headaches
• visual impairment
• paraplegia
• night sweats
• weight loss
• fatigue
• enlarged lymph nodes
• gastrointestinal bleeding
• intestinal perforation

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

• 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.

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.

• 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 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.

• The prognosis for about 90% of pediatric patients and up to 50%-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 is the survival rate for a patient with Burkitt's 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%-60% for adults.
• For adults with low-risk disease, the five-year survival rate is about 71%, while high-risk aggressive lymphomas have about a 29% five-year survival rate.
• The survival rate decreases as the patient's age increases.