Burkitt lymphoma
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The cell of origin is a peripheral IgM+ memory B-cell (presence of somatic hypermutation of the Ig gene)
Epidemiology
Most common in children (1/3 of lymphomas).
3-4% of all lymphomas in adults in western countries
frequently associated with immunodeficiency in adults
Synopsis
endemic variant affecting africans, which primarily involves the jaws and other facial bones.
non-endemic variant may be associated with immunodeficiency and usually presents with abdominal involvement (distal ileum, ciecum, mesentery).
Immunochemistry
Pan-B antigens positive
TdT-
CD10+
CD5-
sIgM+
Cytology
blast cells in the peripheral blood and bone marrow display a basophilic cytoplasm with characteristic vacuolization.
- This aspect is indisinguishable from acute lymphoblastic leukemia (ALL) L3 of the FAB classification, which represents the leukemic counterpart of BL.
Pathology
monomorphic infiltrate of the lymph node by medium-sized cells showing round nuclei with several nucleoli and basophilic cytoplasm.
numerous benign macrophages confer a histologic pattern referred to as ?starry sky? apparance.
Involvement of the peripheral blood and bone marrow may occur.
Localization
nodal Burkitt lymphoma
intestinal Burkitt lymphoma
- ileal Burkitt lymphoma
bilateral mammary Burkitt lymphoma (7761167)
Associations
organ transplantation
- PTLD monotypic Burkitt lymphoma type (12766587)
Differential diagnosis
The related form Burkitt-like lymphoma shows intermediate features between diffuse large cell lymphoma and Burkitt lymphoma and probably includes different disease entities.
- It was suggested by the WHO panel that only those cases with c-MYC rearrangement and/or a >99% proliferation fraction as demonstrated by Ki-67 positivity should be classified as Burkitt-like lymphoma.
Variant
granulomatous reaction in Burkitt lymphoma (correlation with EBV) (16006809, 15104301)
Cytogenetics
8q24 rearrangements (MYC locus)
- translocation t(8;14)(q24;q32) (MYC/IGH) (60-70%)
- translocation t(8;22)(q24;q11) (MYC/) (10-15%)
- translocation t(2;8)(p11;q24) (MYC/) (2-5%)
additional anomalies
- duplication of chromosome 1, involving the 1q21-25 segment as the only detectable chromosome lesion
- 6q11-14 deletion
- 17p deletions (TP53 inactivation)
- trisomy 12, trisomy 7, trisomy 8 and trisomy 18
References
- Duensing S, Lee BH, Dal Cin P, Munger K. Excessive centrosome abnormalities without ongoing numerical chromosome instability in a Burkitt?s lymphoma. Mol Cancer. 2003 Sep 8;2:30. PMID: 14498992
Gong JZ, Stenzel TT, Bennett ER, Lagoo AS, Dunphy CH, Moore JO, Rizzieri DA, Tepperberg JH, Papenhausen P, Buckley PJ. Burkitt lymphoma arising in organ transplant recipients: a clinicopathologic study of five cases. Am J Surg Pathol. 2003 Jun;27(6):818-27. PMID: 12766587
Hammerschmidt W, Sugden B. Epstein-Barr virus sustains Burkitt?s lymphomas and Hodgkin?s disease. Trends Mol Med. 2004 Jul;10(7):331-6. PMID: 15242681