Initial treatment of Waldenström macroglobulinemia

This algorithm describes our preferred management of patients with newly diagnosed WM. There are other acceptable approaches. The clinician is expected to use his or her independent medical judgment in the context of individual circumstances to make adjustments, as necessary.

WM: Waldenström macroglobulinemia; BTK: Bruton tyrosine kinase.
* Indications for treatment:

Recurrent fever, night sweats, weight loss, fatigue
Hyperviscosity
Lymphadenopathy that is either symptomatic or bulky
Symptomatic hepatomegaly and/or splenomegaly
Symptomatic organomegaly and/or organ or tissue infiltration
Peripheral neuropathy due to WM
Symptomatic cryoglobulinemia
Cold agglutinin hemolytic anemia
Immune hemolytic anemia and/or thrombocytopenia
Nephropathy related to WM
Amyloidosis related to WM
Hemoglobin of 10 g/dL or less
Platelet count of 100 × 10⁹/L or less

¶ Symptoms of hyperviscosity include oronasal bleeding, blurred vision, headaches, dizziness, paresthesias, retinal vein engorgement and/or flame-shaped hemorrhages, papilledema, stupor or coma.
Δ Continuous therapy with a BTK inhibitor is an acceptable alternative for patients who decline chemotherapy.

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Initial treatment of Waldenström macroglobulinemia