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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] - 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.
  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