Lymphoma and plasma cell neoplasms
B cell lymphoma subtypes
Burkitt lymphoma

Author: Nikhil Sangle, M.D. (see Authors page)

Revised: 3 April 2017, last major update February 2011

Copyright: (c) 2001-2017, PathologyOutlines.com, Inc.

PubMed Search: burkitt lymphoma

See also Post transplant Burkitt lymphoma
Cite this page: Burkitt lymphoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lymphomaburkitt.html. Accessed July 26th, 2017.
Definition / general
  • High grade B cell lymphoma characterized by CD10+ and c-MYC translocation
Terminology
  • Burkitt leukemia: tumor cells present in blood and diffuse marrow involvement at diagnosis; leukemic cases were previously classified as FAB-L3
  • B cell lymphoma, unclassifiable, with features intermediate between Burkitt lymphoma and diffuse large B cell lymphoma: lymphomas with 2 translocations involving c-MYC and BCL2 or BCL6 (Am J Clin Pathol 2010;134:258)
Epidemiology
  • Either endemic, sporadic or immunodeficiency associated
  • Endemic: tropical Africa, involves jaw or abdomen, 95% are EBV positive (Arch Pathol Lab Med 2004;128:549); malaria may facilitate EBV infection by affecting the EBV-specific T cell response
  • Sporadic: seen throughout the world, mainly in young adults and children (median age of 30 years); high association with EBV in Brazil (Am J Clin Pathol 2008;130:946)
  • Immunodeficiency associated: adults, often HIV+; involves distal ileum, cecum, mesentery
Pathophysiology
  • Due to c-MYC translocation that causes increased constitutive levels of c-MYC
Clinical features
  • 30% of childhood lymphomas
  • Curable with aggressive therapy in 60%
  • Marrow involvement in 15 - 30%
  • May present as B cell acute lymphoblastic leukemia / FAB L3 (also called Burkitt leukemia)
  • Leukemic cases associated with involvement of ileocecum
  • CD5+ Burkitt leukemia: elderly patients with leukemic tumor cells resembling Burkitt lymphoma by morphology and immunostains (Am J Clin Path 1999;112:828); resembles blastoid variant of mantle cell lymphoma (c-MYC negative, cyclin D1+)
Clinical images

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Jaw tumors in African boy

Gross images

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Ileocecal valve tumor,
contributed by Dr. Kaveh Naemi, Irvine, California

Microscopic (histologic) description
  • Diffuse infiltration of monomorphic, medium size (10 - 25 micron) cells with abundant basophilic cytoplasm, noncleaved round nuclei with coarse chromatin and 2 - 5 distinct nucleoli
  • Mitotically active with starry sky pattern (stars are tingible body macrophages)
  • Bone marrow: usually diffuse infiltration of interstitium, with some preservation of adipose tissue; prominent mititoc figures; starry sky feature seen in lymph nodes is rare in marrow
Microscopic (histologic) images

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Ileocecal valve tumor, contributed by Dr. Kaveh Naemi, Irvine, California


CD20+

CD10+

BCL2

Ki67



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Bone marrow
shows medium
sized cells with
cytoplasmic
vacuoles

Starry sky pattern

Burkitt lymphoma
vs. diffuse large
B cell lymphoma
with c-MYC

Ki67 and BCL2
are strongly positive
(uncommon pattern
for BCL2)

Cytology description
Cytology images

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Wright stain

Breast lesion

Positive stains
Negative stains
Flow cytometry description
  • Rare atypical immunophenotypes are negative for SIg light chain, B cell antigens and CD10, and positive for CD4 (Am J Clin Pathol 2010;134:127)
Molecular / cytogenetics description
  • t(8;14)(q24;q32): c-MYC and IgH (75%); also reported in some diffuse large B cell lymphomas, rarely mantle cell lymphoma
  • t(2;8)(p12;q24): Ig kappa and c-MYC (15%); rarely mantle cell lymphoma
  • t(8;22)(q24;q11): c-MYC and Ig lambda (10%)
  • FISH is routinely used for diagnosis, although up to 10% cases lack MYC translocation by FISH; in these cases, PCR or other techniques may be helpful
  • By gene expression profiles, is distinct from other B cell lymphomas (Blood 2011;117:3596)
Molecular / cytogenetics images

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t(8;14)

Differential diagnosis