Diffuse large B-cell lymphoma

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Diffuse large B-cell lymphoma is one of the most common B-cell non-Hodgkin’s lymphomas (B-NHL). It accounts for roughly 40% of all cases of lymphoma, a reflection that the disease label is a broad umbrella for a heterogenous group of high grade lymphomas.

Like the other common B-NHL, follicular lymphoma, diffuse large B-cell lymphomas are dependent on the inflammatory tumour micro-environment for their proliferation and growth. It can be further sub-classified by its tumour genetics which can be correlated with prognosis[1].

Contents

Aetiology

Diffuse large B-cell lymphoma (DLBCL) is associated with a ten times increased risk of prior exposure to Q fever[2] but several other infections are known to predispose, such as Epstein-Barr virus exposure, as well as genotype and interventions such as organ transplantation[3].

Classification

The WHO 'blue book' includes an extensive sub-classification.

The main sub-groups are:

  • Activated B cell-like diffuse large B-cell lymphoma ((ABC) DLBCL, sometimes (non-GCB) DLBCL)
    • Expresses genes that are induced during mitogenic stimulation of B-cells
    • Poorer prognosis, but may respond to NFκB inhibition via bortezomib (REMoDL-B trial in progress as of 2015).
  • Germinal center B-cell-like diffuse large B-cell lymphoma ((GCB) DLBCL)
    • Expresses genes that are hallmarks of normal germinal center B-cells such as BCL-6
    • Best prognosis with classic treatments
  • Primary mediastinal B cell lymphoma (PMBL)
    • First recognised as a rarer lymphoma in young adults
    • 75% share genetic markers with Hodgkins lymphoma, the rest are either (GCB) DLBCL or (ABC) DLBCL

The international prognostic index (IPI) was a clinical tool used to predict prognosis before biologics acting on CD20 were developed.

Treatment

Current multi-agent chemotherapy regimes can cure over 40%.

References

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