Burkitt's lymphoma

Epidemiology

• endemic
  • equatorial Africa
    • most common malignancy of childhood
    • peak 4 - 7 years; male:female 2:1
  • Papua, New Guinea
• sporadic
  • mainly children and young adults
  • 1-2% of all lymphomas in western Europe & USA
  • 30 - 50% of all childhood lymphomas
  • early EBV infection is associated with higher prevalence of EBV positive Burkitt lymphoma
• immunodeficiency associated
  • primarily HIV1 infection
  • EBV identified in ~25-40% of cases

Pathology

• although Burkitt lymphoma may be localized in presentation, it is considered a disseminated disease

working formulation category:

• small noncleaved cell lymphoma, Burkitt's type

Microscopic pathology

• monomorphic medium sized B cells with basophilic cytoplasm and numerous mitotic figures
• variants:
  • classical Burkitt lymphoma
  • Burkitt lymphoma with plasmacytoid differentiation
  • atypical Burkitt/Burkitt-like

Immunophenotype

• panB +, CD10 +, sIgM +, bcl-6 + CD5 -, CD23 -, Tdt -, Bcl-2 -

Genetics

• clonal rearrangements of immunoglobulin heavy & light chain genes
• myc translocation
• p53 incativation in up to 30%
• other implicated genes
  • BCAT1

Clinical manifestations

• neck mass
• night sweats
• splenomegaly
• weight loss
• bulky disease/high tumor burden
• may present as acute leukemia

Laboratory

• serum urate
• serum lactate dehydrogenase

Complications

• airway obstruction
• spontaneous tumor lysis syndrome

Management

• treat as metastatic or disseminated disease, even if disease appears localized
  • cyclophosphamide/vincristine/doxorubicine/dexamethsone, hyper-fractionated + rituximab (R-hyper-CVAD)
  • aggressive hydration & treatment with rasburicase to prevent/treat tumor lysis syndrome
  • neither surgery nor localized radiation beneficial in primary treatment

More general terms

• peripheral B-cell lymphoid neoplasm

More specific terms

• high grade B-cell lymphoma, Burkitt-like

Additional terms

• Burkitt's lymphoma genotyping
• C-X-C chemokine receptor type 5; CXC-R5; CXCR-5; Burkitt lymphoma receptor 1; monocyte-derived receptor 15; MDR-15; CD185 (CXCR5, BLR1, MDR15)
• chromosomal translocation t8q24.1:14q32 (B-cell leukemia, Burkitt's lymphoma)

Patient information

Burkitt's lymphoma patient information