Burkitt's lymphoma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]

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.

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.

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.

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.

Chest, abdomen, and pelvis CT scan may be helpful in the diagnosis of Burkitt’s lymphoma.

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.

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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], Kamal Akbar, M.D.[3]

Burkitt’s lymphoma was first discovered by Denis Parsons Burkitt, an Irish surgeon, in 1958 while working in Africa.

• Burkitt’s lymphoma was first discovered by Denis Parsons Burkitt, an Irish surgeon, in 1958 while working in Africa.^[1]
• In 1961 Burkitt and O’Connor detailed the features of the clinical syndrome known as Burkitt’s lymphoma. Soon afterward, Wright discovered that this lymphoma can be distinguished from other forms of lymphomas based on key histopathological features.^[2]

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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]

Burkitt’s lymphoma may be classified according to the World Health Organization (WHO) classification into 3 clinical variants. The clinical variants are endemic Burkitt’s lymphoma, sporadic Burkitt’s lymphoma, and immunodeficiency-associated Burkitt’s lymphoma.

Burkitt’s lymphoma may be classified according to WHO classification into 3 clinical variants:

• Endemic Burkitt’s lymphoma ^[1]
• Sporadic Burkitt’s lymphoma^[2]
• Immunodeficiency-associated Burkitt’s lymphoma^[3]

It is almost impossible to differentiate these three clinical variants based on histology (i.e. microscopic appearance) or immunophenotype.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Shivali Marketkar, M.B.B.S. [2] Sowminya Arikapudi, M.B,B.S. [3], Kamal Akbar, M.D.[4]

The c-myc gene is 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.

Burkitt’s lymphoma is an aggressive and rapidly growing tumor.^[1]. It can present in a wide variety of locations which include:

• Head and neck (facial bones and Waldeyer’s ring)
• Pleural space (~70%)
• Gastrointestinal tract, especially ileocaecal region
• Mesentery, peritoneum, and retroperitoneum
• Kidneys
• Gonads (~75%)

Translocation of chromosome 8 myc locus with 3 possible partners (accounting for 90% of translocations):^[2]

• The Ig heavy chain region on chromosome 14: t(8;14)
• The kappa light chain locus on chromosome 2: t(2;8)
• The lambda light chain locus on chromosome 22: t(8;22)
• WHO committees suggest the following:

• If morphologic features are intermediate, diagnosis of Burkitt’s should only be made if the Ki-67 fraction of viable cells is at least 99 percent
• If morphologic features suggest diffuse large B cell lymphoma, but have with a high proliferation fraction or t(8;14), they should be classified as diffuse large B cell lymphoma

• Unique genetic alterations promote cell survival in Burkitt’s lymphoma, distinct from other types of lymphoma^[3]
• These TCF3 and ID3 gene mutations in Burkitt’s correspond to a cell survival pathway that may be found to be amenable to targeted therapy.^[4]

• In 2014, it was described that short non-coding RNAs named microRNAs (miRNAs) have important functions in lymphoma biology
• In malignant B cells miRNAs participate in pathways fundamental to B cell development like B cell receptor (BCR) signaling, B cell migration/adhesion, cell-cell interactions in immune niches, and the production and class-switching of immunoglobulins^[5]
• MiRNAs influences B cells in the following manner:^[5]
  • Maturation
  • Generation of marginal zone
  • Follicular
  • Plasma
  • Memory B cells

The tumor cells in Burkitt lymphoma generally strongly express markers of B cell differentiation and they are the following:^[6]

• (CD19
• CD20
• CD22)
• CD10
• BCL6
• The tumor cells are generally negative for BCL2 and TdT. The high mitotic activity of Burkitt’s lymphoma is confirmed by nearly 100% of the cells staining positive for Ki67.^[7]

Malignant B cells have identical DNA recombinations of the V(D)J region of the immunoglobin genes and there characteristics are the following: ^[8]

• This means that no increase in specificity of antibody molecules is occurring in the malignant cells
• These malignant cells are thus clonal populations and can be assayed for by using DNA probes specific for the regions where recombination is expected
• Normal DNA will be characterized by two high concentration of identical germ line DNA V(D)J regions and endless, likely undetectable, non-germline Ig V(D)J DNA
• Lymphoma cells have an additional high concentration of V(D)J DNA that is unlike the germ line, indicating clonal populations of B Cells that are not undifferentiated B cells (germ line DNA cells)
• Assays typically use the process of electrophoresis and southern blot analysis to determine the existence of these characteristics

• 
  Seven-year-old Nigerian boy with a several-month history of jaw swelling which had been treated with antibiotics: The tumor was ulcerated and draining^[9]
• 
  Picture of a mouth of a patient with Burkitt lymphoma showing disruption of teeth and partial obstruction of airway^[9]

On microscopic histopathological analysis, characteristic findings of Burkitt’s lymphoma include:^[10]

• Medium-sized (~1.5-2x the size of a RBC) with uniform size (“monotonous”) — key feature (i.e. tumor nuclei size similar to that of histiocytes or endothelial cells)
• Round nucleus
• Small nucleoli
• Relatively abundant cytoplasm (basophilic)
• Brisk mitotic rate and apoptotic activity
• Cellular outline usually appears squared off
• “Starry-sky pattern”:

• The stars in the pattern are tingible-body macrophages (macrophages containing apoptotic tumor cells)

• The tumour cells are the sky

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Assistant Editor-in-Chief: Soumya Sachdeva, Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]

There are no established causes for Burkitt’s lymphoma.

There are no established causes for Burkitt’s lymphoma.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Assistant Editor-in-Chief: Soumya Sachdeva, Sowminya Arikapudi, M.B,B.S. [2], Kamal Akbar, M.D.[3]

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 must be differentiated from other diseases such as:^[1]

• Diffuse large B cell lymphoma
• Hodgkin’s lymphoma
• Follicular lymphoma
• Mucosa-Associated Lymphatic Tissue lymphoma (MALT)
• Small cell lymphocytic lymphoma
• Mantle cell lymphoma (MCL)

Endemic Burkitt’s lymphoma typically presents as a cervico-facial mass and must be differentiated from other diseases that may present as a cervico-facial mass such as salivary glands tumors, sialolithiasis, human immunodeficiency virus, radiation, and systemic diseases such as, sarcoidosis, and sjögren’s syndrome.^{[25][26][27][28][29][30][31][32]}

Based on the expression of cell surface markers, the table below differentiates Burkitt’s lymphoma from other diseases that cause similar clinical presentations:^[33]

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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], Kamal Akbar, M.D.[3]

Burkitt’s lymphoma is a common disease that tends to affect children or young adults. Males are more commonly affected with Burkitt’s lymphoma compared to females. Burkitt’s lymphoma usually affects individuals of the African race.

• The incidence of is approximately 3 to 6 cases per 100,000 children per year for endemic and 0.02 cases per 100,000 people for sporadic burkitt’s lymphoma^[1][2]

• Burkitt’s lymphoma is a common disease that tends to affect children or young adults. The median age at diagnosis is 8 years^[3]

• Males are more commonly affected with Burkitt’s lymphoma than females. The Male to Female ratio is approximately 4 to 1^[4]

• Burkitt’s lymphoma usually affects individuals of the African race^[4]

• The majority of Burkitt’s lymphoma cases are reported in equitorial Africa^[5]

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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], Kamal Akbar, M.D.[3]

Common risk factors in the development of Burkitt’s lymphoma are HIV/AIDS and post-transplant immunosuppression. There are no established risk factors for childhood Burkitt’s lymphoma.

Common risk factors in the development of Burkitt’s lymphoma may include:^[1]

• HIV/AIDS^[2]
• Post-transplant immunosuppression^[3]
• Solid organ transplant^[4]
• Malaria^[1]

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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]

According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for Burkitt’s lymphoma.

According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for Burkitt’s lymphoma.^[1]

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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], Kamal Akbar, M.D.[3]

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.

Common complications of Burkitt’s lymphoma include:

• Disruption of teeth^[1]
• Partial obstruction of airway^[2]
• Ulceration and drainage^[3]
• Tumor lysis syndrome^[4]

• In children, prognosis is good with survival rates >90%^[5]
• In adults, prognosis is poor, with a 5-year survival rate of 50% and is even worse with bone marrow or CNS involvement (>30% 5-year survival rate).

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