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Burkitt's lymphoma

Epidemiology: 1) endemic - equatorial Africa - most common malignancy of childhood - peak 4 - 7 years; male:female 2:1 - Papua, New Guinea 2) 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 3) 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 Genetics: - clonal rearrangements of immunoglobulin heavy & light chain genes - myc translocation - p53 incativation in up to 30% - other implicated genes - BCAT1 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 - 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 [3] Complications: - airway obstruction - spontaneous tumor lysis syndrome [3,5] Management: - treat as metastatic or disseminated disease, even if disease appears localized [3] - cyclophosphamide/vincristine/doxorubicine/dexamethsone, hyper-fractionated + rituximab (R-hyper-CVAD) [3] - aggressive hydration & treatment with rasburicase to prevent/treat tumor lysis syndrome [3] - diuretic only as needed to maintain urine output or if volume overload [3] - neither surgery nor localized radiation beneficial in primary treatment [3]

Interactions

disease interactions

Related

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)

Specific

high grade B-cell lymphoma, Burkitt-like

General

peripheral B-cell lymphoid neoplasm

References

  1. WHO Classification Tumours of Haematopoietic and Lymphoid Tissues. IARC Press 2001.
  2. Molyneux EM, Rochford R, Griffin B et al Burkitt's lymphoma. Lancet. 2012 Mar 31;379(9822):1234-44 PMID: 22333947
  3. Medical Knowledge Self Assessment Program (MKSAP) 17, 18, 19. American College of Physicians, Philadelphia 2015, 2018, 2021. - Medical Knowledge Self Assessment Program (MKSAP) 20 American College of Physicians, Philadelphia 2025
  4. Casulo C, Friedberg J. Treating Burkitt Lymphoma in Adults. Curr Hematol Malig Rep. 2015 Sep;10(3):266-71. Review. PMID: 26013028
  5. NEJM Knowledge+ Hematology
  6. Crombie J, LaCasce A The treatment of Burkitt lymphoma in adults. Blood. 2021 Feb 11;137(6):743-750. PMID: 33171490 Free article. Review https://www.sciencedirect.com/science/article/pii/S0006497121002615