Waldenstrom’s Macroglobulinaemia Management

Some Salient Points:-

  1. Asymptomatic WM cases don’t require emergent treatment and can be followed up.
  2. 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. 
  3. 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.
  4. R-Bendamustine seems to be the preferred regimen when non-BTK inhibitor-based therapy is chosen.
  5. When there is baseline neuropathy (even subclinical) in the patient, then BDR (Bort-Dexa-Rituxi) should be avoided.
  6. If the disease is bulky or presents with symptomatic hyperviscosity then RCD/DRC is not a good option.
  7. Plasma Exchange must be offered along with starting chemotherapy in patients with symptomatic hyperviscosity.
  8. BTK inhibitors work best in MYD88MUT/CXCR4WT patients.
  9. 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)


Leave a Reply