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Published in final edited form as: Annu Rev Immunol. 2012 Jan 6;30:565–610. doi: 10.1146/annurev-immunol-020711-075027

Summary of clinical, pathological, and molecular characteristics of mature B cell malignancies. (a) The major lymphoma subtypes discussed in this review are listed with their approximate frequency among B cell neoplasms indicated. See text for details. (b) Transcription factor networks in normal and malignant B cells. The most similar normal B cell counterpart for each malignancy is shown. Follicular lymphoma, Burkitt lymphoma, and germinal center B cell–like (GCB) diffuse large B cell lymphoma (DLBCL) have gene expression profiles that resemble those of normal germinal center B cells. A suite of transcription factors are required to generate and/or maintain normal germinal center B cells. These factors control the indicated cellular processes, which favor the proliferation and selection of germinal center B cells bearing B cell receptors (BCRs) with high affinity for antigen while promoting the apoptosis of B cells with low-affinity BCRs. Activated B cell–like (ABC) DLBCL resembles the post-germinal center plasmablast, a normally transient state en route to terminal plasmacytic differentiation. Constitutive NF-κB activity in ABC DLBCL upregulates expression of the transcription factor IRF4, which drives plasmacytic differentiation. Terminal differentiation is blocked at the plasmablast stage in ABC DLBCL by inactivation of the transcription factor Blimp-1 (see text for details).

Summary of clinical, pathological, and molecular characteristics of mature B cell malignancies. (a) The major lymphoma subtypes discussed in this review are listed with their approximate frequency among B cell neoplasms indicated. See text for details. (b) Transcription factor networks in normal and malignant B cells. The most similar normal B cell counterpart for each malignancy is shown. Follicular lymphoma, Burkitt lymphoma, and germinal center B cell–like (GCB) diffuse large B cell lymphoma (DLBCL) have gene expression profiles that resemble those of normal germinal center B cells. A suite of transcription factors are required to generate and/or maintain normal germinal center B cells. These factors control the indicated cellular processes, which favor the proliferation and selection of germinal center B cells bearing B cell receptors (BCRs) with high affinity for antigen while promoting the apoptosis of B cells with low-affinity BCRs. Activated B cell–like (ABC) DLBCL resembles the post-germinal center plasmablast, a normally transient state en route to terminal plasmacytic differentiation. Constitutive NF-κB activity in ABC DLBCL upregulates expression of the transcription factor IRF4, which drives plasmacytic differentiation. Terminal differentiation is blocked at the plasmablast stage in ABC DLBCL by inactivation of the transcription factor Blimp-1 (see text for details).

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