NUCLEAR FACTOR KAPPA-LIGHT-CHAIN-ENHANCER OF ACTIVATED B CELLS (NF-KB)

Molecular Evidence (rationale) for NF-kB targeted therapy in iMCD patients:

• “NF-kB is the primary transcription factor for several of the cytokines commonly elevated in iMCD, and it has been proposed as one of the important dysregulated pathways in iMCD pathogenesis” (Blood 2014)

Case Reports of anti-NF-kB therapy for iMCD patients:

Bortezomib (Velcade) is “a selective proteasome inhibitor that preferentially targets plasma cells, has lowered IL-6 levels and induced remission in 4 cases of iMCD” (Blood 2014) “Bortezomib may work in iMCD via direct inhibition of NF-kB” (Blood 2014)

• Please click here to read case reports of iMCD patients receiving Bortezomib.

Corticosteroids: “Many of the immunosuppressive actions of glucocorticoids are mediated by interference with signalling by the key inflammatory transcriptional regulators; NF-κB” (Coutinho and Chapman)

• Please click here to read case reports of iMCD patients receiving corticosteroid monotherapy.

Clinical Trials targeting NF-kB in iMCD:

No clinical trials targeting NF-kB in iMCD patients have been performed.