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  • Evaluation of pretreatment prognostic factors of diffuse large B-cell lymphoma (DLBCL) includes scores such as the International Prognostic Index (IPI); cell-of-origin (germinal center B-cell subtype [GCB] and activated B-cell [ABC] subtype); genetic profiling (such as fluorescence in situ hybridization studies [FISH] for MYC rearrangement, if MYC rearrangement is detected, additional FISH studies for BCL2 and BCL6 rearrangements should be performed); and immunophenotypic studies (such as MYC, BCL2, and BCL6 expression).

  • The baseline workup of aggressive B-cell lymphoma should not omit testing for hepatitis B and HIV, echocardiogram, or multigated acquisition scan (if planned therapy with anthracycline), and fertility counseling for eligible patients.

  • Besides a clinical trial, the standard-of-care frontline therapy of DLBCL not otherwise specified is still rituximab cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP). However, many studies are ongoing to outperform this combination.

  • The treatment plan of aggressive B-cell lymphoma should evaluate the potential need for central nervous system (CNS) prophylaxis for patients presenting with the following risk factors: elevated CNS-IPI (age >60 years, Eastern Cooperative Oncology Group [ECOG] Performance Status ≥2, extranodal disease >1 site, stage III/IV, elevated lactate dehydrogenase, kidney/adrenal involvement); extranodal disease with testicular, uterine, breast, epidural, skin involvement); aggressive B-cell lymphoma subtypes (Burkitt lymphoma, double/triple-hit lymphoma/high-grade B-cell lymphoma, double-expressor lymphomas, HIV-associated lymphoma, DLBCL with ABC subtype, intravascular DLBCL, CD5+ DLBCL, IgM-secreting DLBCL).

  • The baseline workup of a suspected primary CNS lymphoma should include magnetic resonance imaging (MRI) of the brain and biopsy of the lesion, search for additional disease sites, such as testicular ultrasound for men >60 years old, full ophthalmologic examination including slit-lamp eye examination, spine MRI, positron emission tomography/computed tomography (PET/CT) scan, and bone marrow biopsy.

  • The optimal frontline therapy of Burkitt lymphoma has yet to be defined in a prospective randomized trial, but R-CHOP is not an adequate therapy. If a patient cannot be enrolled in a clinical trial, aggressive combination chemotherapy such as R-Hyper-CVAD/MA, R-CODOX-M/IVAC, or DA-EPOCH-R with adequate CNS prophylaxis is recommended.


It is clinically useful to divide B-cell non-Hodgkin lymphomas (NHLs) into indolent and aggressive groups based on their clinical behavior.1 Patients with indolent NHLs typically have a survival of many years, even if the NHL is untreated, but paradoxically, the NHLs are usually incurable. Patients with aggressive NHL have a survival time measured in weeks to months if untreated yet the NHLs are usually chemosensitive and frequently curable. In this chapter, we focus on the clinical characteristics, pathology, and treatment of aggressive B-cell NHLs.


Globally, lymphoma incidence rates have declined slightly (0.3% per year) since 2001 in females and since 2004 in males in the US. Earlier, lymphoma incidence rates increased steadily during the 1970s and 1980s, leveled off in the 1990s, and began a slight decline by the end of that decade. This drop, independent of HIV infection may in part reflect International Classification of Diseases coding changes in 2001, when the ...

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