Burkitt's lymphoma overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Burkitt's lymphoma (or "Burkitt's tumor", or "Malignant lymphoma, Burkitt's type") is a cancer of the lymphatic system (in particular, B lymphocytes). It is associated with the Epstein-Barr virus, also the cause of mononucleosis as well as other cancers. It is named after Denis Parsons Burkitt, a surgeon who first described the disease in 1956 while working in equatorial Africa.
Children affected with the disease often also had chronic malaria which is believed to have reduced resistance to the Epstein-Barr virus and allowed it to take hold. This is known as classical African or endemic Burkitt's lymphoma. Disease characteristics include large tumors in the facial or abdominal regions.
The sporadic type of Burkitt's lymphoma (also known as "non-African") is another form of non-Hodgkin lymphoma found outside of Africa in which cancer cells have a similar appearance to the cancer cells of classical African or endemic Burkitt's lymphoma. Again it is believed that impaired immunity provides an opening for development of the Epstein-Barr virus. Examination of chromosomes from these tumor cells often shows translocation of the myc and Ig genes (t: 8;14).
Historical Perspective
Burkitt's lymphoma was first described by Denis Parsons Burkitt, a surgeon, in 1958 while working in equatorial Africa.
Classification
Burkitt's lymphoma may be classified according to WHO classification into 3 clinical variants: endemic Burkitt's lymphoma, sporadic Burkitt's lymphoma, and immunodeficiency-associated Burkitt's lymphoma.
Pathophysiology
Gene involved in the pathogenesis of Burkitt's lymphoma includes c-myc. On gross pathology, ulceration and discharge are characteristic findings of Burkitt's lymphoma. On microscopic histopathological analysis, "starry sky" appearance is characteristic finding of Burkitt's lymphoma.
Causes
There are no established causes for Burkitt's lymphoma.
Differential diagnosis
Burkitt's lymphoma must be differentiated from other diseases such as Hodgkin's lymphoma, diffuse large B cell lymphoma, follicular lymphoma, Mucosa-Associated Lymphatic Tissue lymphoma (MALT), small cell lymphocytic lymphoma, and mantle cell lymphoma (MCL).
Epidemiology and Demographics
Burkitt's lymphoma is a common disease that tends to affect children or young adults. Males are more commonly affected with Burkitt's lymphoma than females. Burkitt's lymphoma usually affects individuals of the African race.
Risk Factors
Common risk factors in the development of Burkitt's lymphoma may be HIV/AIDS and post-transplant immunosuppression.
Screening
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for Burkitt's lymphoma.
Prognosis
The prognosis varies with the age, children have the most favorable prognosis. Burkitt's lymphoma in children is associated with a 5 year survival rate of 90%. The presence of metastasis in bone marrow or CNS is associated with a particularly poor prognosis among patients with Burkitt's lymphoma.
Diagnosis
Staging
According to the Murphy staging system, there are four stages of Burkitt's lymphoma based on the number of nodes and extra nodal involvement.
Laboratory Findings
Laboratory tests for Burkitt's lymphoma include complete blood count (CBC), blood chemistry studies, immuno histochemistry, HIV blood test, flow cytometric analysis, and cytogenetics.
X Ray Findings
Chest and knee x ray may be helpful in the diagnosis of Burkitt's lymphoma.
CT
Chest, abdomen, and pelvis CT scan may be helpful in the diagnosis of Burkitt's lymphoma.
MRI
MRI scan may be helpful in the diagnosis of Burkitt's lymphoma.
Biopsy
Lymph node or extra nodal tissue biopsy is diagnostic of Burkitt's lymphoma.
Other Imaging Studies
PET scan and bone scan may be helpful in the diagnosis of Burkitt's lymphoma.
Other diagnostic studies
Other diagnostic studies for the diagnosis of Burkitt's lymphoma include bone marrow biopsy and spinal fluid examination.
Treatment
Medical Therapy
The predominant therapy for Burkitt's lymphoma is chemotherapy. Adjunctive immunotherapy and stem cell transplantation may be required.
Surgery
Surgical intervention is not recommended for the management of Burkitt's lymphoma.