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Lymphoma - B cell neoplasms

B cell lymphoma subtypes

Unclassifiable, features between Burkitt and diffuse large B cell lymphoma


Reviewer: Nikhil Sangle, M.D., University of Utah and ARUP Laboratories (see Reviewers page)
Revised: 8 February 2011, last major update February 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

Definition
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● New entity (2008) with features intermediate between Burkitt lymphoma and diffuse large B cell lymphoma
● Includes cases with:
- (1) proliferation index > 90% and "starry sky" appearance, but marked variation in cytomorphology not acceptable for Burkitt lymphoma
- (2) uniform medium sized cells but not the typical immunophenotype of Burkitt lymphoma, such as strong BLC2 staining or proliferation index < 90%, or
- (3) transformations of prior low grade B cell lymphoma with MYC rearrangement (J Hematop 2009 Jun 27 [Epub ahead of print])

Terminology
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● New entity in 2008 WHO classification
● Complete name is: B cell lymphoma, unclassifiable, with features intermediate between diffuse large B cell lymphoma and Burkitt lymphoma
● Also called "double hit" lymphoma
● Includes the prior entity of Burkitt-like or atypical Burkitt lymphoma

Pathophysiology
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● Includes Burkitt-like transformation of follicular lymphoma
● Aggressive nature likely reflects their simultaneous expression of both pro-proliferative (MYC) and anti-apoptotic (bcl2) oncoproteins

Clinical features
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● Usually adults, with aggressive disease (Am J Surg Pathol 2010;34:327)
● Rarely presents as lymphomatoid polyposis of GI tract (J Gastrointest Cancer 2011 Jan 7 [Epub ahead of print])

Micro description
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● Cellular pleomorphism and heterogeneity intermediate between Burkitt lymphoma and diffuse large B cell lymphoma

Flow cytometry description
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● Decreased expression of CD20 (Am J Clin Pathol 2010;134:258)

Molecular description
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● Often "double hit" lymphomas, with MYC rearrangements with non-IGH genes, with a complex karyotype possibly involving bcl2 and, less frequently, bcl6 rearrangements (Blood 2010 Nov 30 [Epub ahead of print])

End of Lymphoma - B cell neoplasms > Unclassifiable, features between Burkitt and diffuse large B cell lymphoma


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