Burkitt lymphoma

Burkitt lymphoma is a rare, highly aggressive B-cell lymphoma that is most common in children and adolescents. It is classified into three variants, each with different epidemiological and clinical characteristics. Presentation is typically dramatic, with a rapidly growing mass that spreads systemically. Gastrointestinal tract involvement is common, especially in the sporadic variant. Management includes systemic chemoimmunotherapy, with a remission rate of 90% in children and adolescents.

Burkitt lymphoma is classified into three variants, each with different epidemiological and clinical characteristics. ^[1][2][3]

• Sporadic variant
  • Mostly affects children and adolescents, and adults > 70 years old. ^[3]
  • Variant that most commonly affects the GI tract.
  • May be associated with EBV infection, especially in older adults
• Endemic variant
  • Mostly affects children and adolescents
  • 95–100% of patients have EBV infection; infection with Plasmodium spp. is also common. ^[3]
• Immunodeficiency-associated variant
  • Mostly affects adults
  • Commonly associated with HIV infection or EBV infection

• Burkitt lymphoma accounts for up to 50% of all childhood lymphomas, depending on the variant. ^[3]
• The sporadic and endemic variants most commonly affect children and adolescents; the immunodeficiency-associated variant typically affects adults. ^[1][3]
• The endemic variant occurs in equatorial Africa and Papua New Guinea. ^[3]

Burkitt lymphoma is most common in children and adolescents. ^[3]

Epidemiological data refers to the US, unless otherwise specified.

• Derived from germinal center B cells ^[1][2]
• t(8;14) leads to overactivation of the C-MYC gene and transcription. ^[1][2]

Presentation is typically dramatic, with a rapidly growing mass that spreads systemically. ^[1][2][3]

• GI tract involvement may occur in all variants (most common in the sporadic variant)
  • Rapidly growing large masses, commonly in the ileocecal region
  • Bowel obstruction
• Systemic involvement
  • Peritoneum
  • Thyroid
  • Breasts
  • Kidney
  • Ovaries, testicles
  • Lymphnodes and bone marrow (especially in the immunodeficiency-associated variant))
  • CNS
• Complications
  • Tumor lysis syndrome
  • Hemorrhage, necrosis
  • Intestinal perforation

• Diffuse large B-cell lymphoma
• Other high-grade B-cell lymphomas with MYC translocations, e.g.:
  • Mantle cell lymphoma
  • Precursor B-cell lymphoblastic lymphoma

The differential diagnoses listed here are not exhaustive.

General principles ^[3]

• Obtain initial laboratory studies (e.g., CBC, LDH, viral serologies).
• Staging of non-Hodgkin lymphomas is required for all patients.
• Diagnosis is confirmed with histopathological studies.
• See "Diagnosis of non-Hodgkin lymphoma" for more information.

Histopathology ^[1][2][3]

A large-bore core needle or surgical biopsy is required; findings are similar across the three variants of Burkitt lymphoma.

• Diffuse infiltrate of monomorphic, medium-sized B cells
  • Round nuclei with vesicular chromatin and multiple basophilic nucleoli
  • Basophilic cytoplasm
• Ki-67 index ∼ 100% ^[1][3]
• Multiple tingible body macrophages ("starry sky" pattern)

Immunohistochemistry ^[1][2][3]

• Positive markers
  • B-cell markers (e.g., CD20)
  • Germinal center B-cell markers: CD19, CD10, BCL-6, PAX5
• Negative markers
  • BCL-2
  • CD5
  • CD23

Cytogenetics

• MYC t(8;14) in ∼ 75% of tumors ^[1][3]
• May not be detected with standard fluorescence in situ hybridization

Emergency management is often required due to the high rate of complications. ^[3]

• Systemic chemoimmunotherapy: combination regimens (e.g., cyclophosphamide, rituximab, methotrexate) based on patient risk
• CNS prophylaxis: in patients with features of high-risk tumors (e.g., patients with ECOG PS ≥ 2, ↑ LDH, tumor size ≥ 7 cm)
• Management of complications (e.g., management of tumor lysis syndrome).
• See also "Treatment of non-Hodgkin lymphoma."

Burkitt lymphoma can be cured in ∼ 90% of children and adolescents. ^[3]