A 52-year-old woman is diagnosed with Waldenström's macroglobulinemia. She has hyperviscosity symptoms (blurred vision, headache). IgM level is 6,200 mg/dL. Bone marrow shows lymphoplasmacytic lymphoma. MYD88 L265P mutation is detected. Which is the most appropriate immediate therapy?
- A Ibrutinib (BTK inhibitor) monotherapy
- B Plasmapheresis to reduce serum viscosity urgently ✓
- C High-dose dexamethasone only
- D Rituximab monotherapy immediately
Correct answer: B. Plasmapheresis to reduce serum viscosity urgently
Explanation
Symptomatic hyperviscosity syndrome from Waldenström's macroglobulinemia requires urgent plasmapheresis (plasma exchange) to rapidly reduce IgM levels and viscosity, preventing stroke and retinal hemorrhage. MYD88 L265P mutation is present in >90% of Waldenström's and confirms diagnosis. Ibrutinib is effective long-term treatment but does not act rapidly enough for acute hyperviscosity. Rituximab may paradoxically worsen IgM flare (IgM flare phenomenon) and should not be used alone in acute hyperviscosity.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.