Lymphoma & related disorders
Mature B cell neoplasms
DLBCL and large B cell lymphomas with high grade features
Burkitt lymphoma
Author: Nikhil Sangle, M.D.
Topic Completed: 1 February 2011
Minor changes: 8 December 2020
Copyright: 2001-2021, PathologyOutlines.com, Inc.
PubMed Search: burkitt lymphoma
See Also: Post transplant Burkitt lymphoma
Table of Contents
Definition / general | Terminology | Epidemiology | Pathophysiology | Clinical features | Clinical images | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Cytology images | Positive stains | Negative stains | Flow cytometry description | Molecular / cytogenetics description | Molecular / cytogenetics images | Differential diagnosisCite this page: Sangle N. Burkitt lymphoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphomaburkitt.html. Accessed January 22nd, 2021.
Definition / general
- High grade B cell lymphoma characterized by CD10+ and c-MYC translocation
- See also prior bone topic (topics are being merged)
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
Images hosted on other servers:
Jaw tumors in African boy
Gross images
Contributed by Dr. Kaveh Naemi
Ileocecal valve tumor
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
Contributed by Dr. Kaveh Naemi
Ileocecal valve tumor
CD20+
CD10+
BCL2
Ki67
Images hosted on other servers:
Starry sky pattern
Cytology description
- Imprints demonstrate cytoplasmic lipid vacuoles; rarely granulomas (Am J Surg Pathol 2004;28:379)
Cytology images
Images hosted on other servers:
Wright stain
Breast lesion
Positive stains
- CD10, CD19, CD20, Ki67 / MIB1 (almost 100%), surface immunoglobulin
- Also CD22, CD33 (myeloid antigen, J Clin Pathol 1993;46:778), CD43, CD79a, BCL6, surface IgM
- EBV positive in endemic African cases and AIDS cases
- Nuclear staining with new c-MYC antibody (Am J Surg Pathol 2010;34:882)
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
Images hosted on other servers:
t(8;14)
Differential diagnosis
- CD10+ diffuse large B cell lymphoma with c-MYC translocation: higher levels of CD44 and CD54 by flow cytometry (Am J Clin Pathol 2010;133:718)
- Diffuse large B cell lymphoma with c-MYC translocation: more pleomorphic cells, c-MYC negative (usually), Ki67+ in 67% vs. 100% in Burkitt (Mod Pathol 2002;15:771)
- Pre B lymphoblastic lymphoma: usually present as leukemia, no surface immunoglobulin, TdT+, c-MYC negative
