Some Salient Points:-
- Asymptomatic WM cases don’t require emergent treatment and can be followed up.
- IgM MGUS cases associated with Peripheral Neuropathy (without fitting into WM/IgM MM criteria) require treatment provided other causes of neuropathy has been ruled out by thorough neurological examination and tests and neuropathy is attributable to IgM MGUS reasonably.
- The level of monoclonal IgM alone is not an indication to start treatment. However, IgM levels >60 g/L are associated with an imminent risk of symptomatic hyperviscosity and are therefore considered to be a treatment indication.
- R-Bendamustine seems to be the preferred regimen when non-BTK inhibitor-based therapy is chosen.
- When there is baseline neuropathy (even subclinical) in the patient, then BDR (Bort-Dexa-Rituxi) should be avoided.
- If the disease is bulky or presents with symptomatic hyperviscosity then RCD/DRC is not a good option.
- Plasma Exchange must be offered along with starting chemotherapy in patients with symptomatic hyperviscosity.
- BTK inhibitors work best in MYD88MUT/CXCR4WT patients.
- In patients with MYD88WT it is preferable to use R-Chemo (eg R-Benda) when compared to Ibrutinib. If R-Chemo is not feasible in such patients then it is preferable to add Rituximab to Ibrutinib (ie R-Ibrutinib)