Home   Chapter Home   Jobs   Conferences   Fellowships   Books



Advertisement

Lymphoma - B cell neoplasms

B cell lymphoma subtypes

Burkitt’s lymphoma


Reviewer: Nikhil Sangle, M.D., University of Utah and ARUP Laboratories (see Reviewers page)
Revised: 7 February 2012, last major update February 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

See also Post-transplant Burkitt's lymphoma

Definition
=========================================================================

● High grade B cell lymphoma characterized by CD10+ and c-myc translocation

Terminology
=========================================================================

Burkitt’s 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’s leukemia)
● Leukemic cases associated with involvement of ileocecum
CD5+ Burkitt's leukemia: elderly patients with leukemic tumor cells resembling Burkitt's 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
=========================================================================


   
Jaw tumors in African boys

Gross images
=========================================================================



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

Micro description
=========================================================================

● Diffuse infiltration of monomorphic, medium-sized (10-25 micron) cells with abundant basophilic cytoplasm, non-cleaved 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

Micro images
=========================================================================



Bone marrow shows medium sized cells with cytoplasmic vacuoles

       
Starry sky pattern

           
Contributed by Dr. Kaveh Naemi, Irvine, California - ileocecal valve tumor


Burkitt's lymphoma versus diffuse large B cell lymphoma with c-myc

   
CD20+                         CD10+

   
bcl2                              Ki-67


Ki-67 and bcl2 are strongly positive (uncommon pattern for bcl2)

Positive stains
=========================================================================

● CD10, CD19, CD20, Ki-67/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)

Negative stains
=========================================================================

● CD5, CD23, TdT, bcl2 (positive in 20%)

Flow cytometry
=========================================================================

● 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)

Cytology description
=========================================================================

● Imprints demonstrate cytoplasmic lipid vacuoles; rarely granulomas (Am J Surg Pathol 2004;28:379)

Cytology images
=========================================================================



Wright's stain


Breast lesion

Molecular 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 Jan 18 [Epub ahead of print])

Molecular images
=========================================================================



t(8;14)

Differential diagnosis
=========================================================================

PreB lymphoblastic lymphoma: usually present as leukemia, no surface immunoglobulin, TdT+, c-myc negative
Diffuse large B cell lymphoma with c-myc translocation: more pleomorphic cells, c-myc negative (usually), Ki67+ in 67% vs. 100% in Burkitt’s (Mod Pathol 2002;15:771)
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)

End of Lymphoma - B cell neoplasms > B cell lymphoma subtypes > Burkitt’s lymphoma


This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions (click here for other contact information).