Burkitt's lymphoma overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]
Overview
Burkitt's lymphoma ("Burkitt's tumor", or "Malignant lymphoma, Burkitt's type") is a cancer of the lymphatic system (in particular, B lymphocytes). Burkitt's lymphoma was first described by Denis Parsons Burkitt, a surgeon, in 1958 while working in equatorial Africa. 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. 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. There are no established causes for Burkitt's lymphoma. 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). 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. Common risk factors in the development of Burkitt's lymphoma may be HIV/AIDS and post-transplant immunosuppression. According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for Burkitt's lymphoma. 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. 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 tests for Burkitt's lymphoma include complete blood count (CBC), blood chemistry studies, immuno histochemistry, HIV blood test, flow cytometric analysis, and cytogenetics. Chest and knee x ray may be helpful in the diagnosis of Burkitt's lymphoma. CT and MRI may be helpful in the diagnosis of Burkitt's lymphoma. Lymph node or extra nodal tissue biopsy is diagnostic of Burkitt's lymphoma. PET and bone scan may be helpful in the diagnosis of Burkitt's lymphoma. Other diagnostic studies for the diagnosis of Burkitt's lymphoma include bone marrow biopsy and spinal fluid examination. The predominant therapy for Burkitt's lymphoma is chemotherapy. Adjunctive immunotherapy and stem cell transplantation may be required. Surgical intervention is not recommended for the management of Burkitt's lymphoma.
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
The c-myc gene involved in the pathogenesis of Burkitt's lymphoma. On gross pathology, ulceration and discharge are characteristic findings of Burkitt's lymphoma. On microscopic histopathological analysis, "starry sky" appearance is a 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 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 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.