B-cell Lymphoma, Unclassifiable, with Features Intermediate Between Diffuse Large B-cell Lymphoma and Burkitt Lymphoma



B-cell Lymphoma, Unclassifiable, with Features Intermediate Between Diffuse Large B-cell Lymphoma and Burkitt Lymphoma


Carlos E. Bueso-Ramos, MD, PhD










B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and BL (DLBCL/BL). Note the high number of apoptotic cells and the mixture of intermediate and large-sized cells.






Conventional cytogenetic analysis of a case of DCBCL/BL shows a complex karyotype. The presence of t(8;14) (q24;q32) image and t(14;18)(q32;q21) image supports further classification as a “double-hit” lymphoma.


TERMINOLOGY


Abbreviations



  • B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma (BL)


Synonyms



  • Burkitt-like lymphoma


  • High-grade B-cell lymphoma, Burkitt-like (Revised European American Lymphoma [REAL] classification)


  • Small noncleaved cell lymphoma, non-Burkitt (Working Formulation)


Definitions



  • Aggressive B-cell lymphoma with morphological and genetic features of both DLBCL and BL


  • World Health Organization (WHO) classification considers this category



    • Heterogeneous and not distinct entity


    • Useful as “parking lot” for unclassifiable high-grade B-cell lymphomas


ETIOLOGY/PATHOGENESIS


Unknown



  • Subset of DLBCL/BL cases includes “double-hit” or “triple-hit” lymphomas



    • “Hit” in this context refers to translocations involving MYC, BCL2, or BCL6 genes


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Uncommon, but true frequency not yet known



      • Frequency increases with patient age


  • Age



    • Adults; median: 6th decade


  • Gender



    • Males > females


Site



  • > 50% of patients present with disseminated disease



    • Lymph nodes


    • Extranodal sites common and include



      • Bone marrow (˜ 50%) and brain (˜ 33%)


Presentation



  • Patients present with lymphadenopathy &/or extranodal masses


  • ˜ 10-20% of patients have history of follicular lymphoma


  • B-type symptoms common


  • Leukemic presentation uncommon but can occur


Laboratory Tests



  • Elevated serum lactate dehydrogenase &/or β-2-microglobulin levels


  • Elevated leukocyte count with lymphoma cells in patients with leukemic presentation


Natural History



  • Aggressive clinical course for most patients


  • Poor prognostic factors



    • High clinical stage


    • Central nervous system involvement


    • History of follicular lymphoma


    • Double- or triple-hit lymphoma


Treatment



  • Drugs



    • No consensus on optimal therapeutic approach


    • Rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) usually not effective


    • Rituximab, hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone (R-HyperCVAD) effective for subset of patients



Prognosis



  • DLBCL/BL is heterogeneous and therefore prognosis is also heterogeneous


  • Large subset of patients have poor clinical outcome despite rigorous chemotherapy regimen


IMAGE FINDINGS


CT Findings



  • F-18 fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT)



    • DLBCL/BL is highly FDG avid

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on B-cell Lymphoma, Unclassifiable, with Features Intermediate Between Diffuse Large B-cell Lymphoma and Burkitt Lymphoma
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