Drug1st line

Bortezomib (BDR regimen)

Proteasome inhibitor (with dexamethasone + rituximab)

Response rate
~80-85% (frontline BDR)
Onset
Weeks-months
Route
SC 1.3mg/m2 (weekly) + dexamethasone + rituximab
Line
1st
IgM effect
Significant reduction
Evidence level
amber

Evidence summary

Proteasome inhibitor active against plasma cell component of WM. Used in BDR (bortezomib-dexamethasone-rituximab) combination. Effective in both frontline and relapsed settings. Risk of peripheral neuropathy limits use, especially in patients with pre-existing IgM neuropathy. Subcutaneous route reduces neuropathy risk.

Approved indications

Conditions for which Bortezomib (BDR regimen) has regulatory approval (not specific to rare diseases covered here):

Multiple MyelomaMantle Cell Lymphoma

Drug identifiers

DrugBankDB00188
ATC CodeL01XG01