Table 4.

Indications to start therapy in a patient with a diagnosis of WM

Clinical indications for initiation of therapy
Recurrent fever, night sweats, weight loss, fatigue 
Hyperviscosity 
Lymphadenopathy: either symptomatic or bulky (≥5 cm in maximum diameter) 
Symptomatic hepatomegaly and/or splenomegaly 
Symptomatic organomegaly and/or organ or tissue infiltration 
Peripheral neuropathy because of WM 
Clinical indications for initiation of therapy
Recurrent fever, night sweats, weight loss, fatigue 
Hyperviscosity 
Lymphadenopathy: either symptomatic or bulky (≥5 cm in maximum diameter) 
Symptomatic hepatomegaly and/or splenomegaly 
Symptomatic organomegaly and/or organ or tissue infiltration 
Peripheral neuropathy because of WM 
Laboratory indications for initiation of therapy
Symptomatic cryoglobulinemia 
Symptomatic cold agglutinin anemia 
Autoimmune hemolytic anemia and/or thrombocytopenia 
Nephropathy that is related to WM 
Amyloidosis that is related to WM 
Hemoglobin ≤10 g/dL 
Platelet count <100 × 109/L 
Laboratory indications for initiation of therapy
Symptomatic cryoglobulinemia 
Symptomatic cold agglutinin anemia 
Autoimmune hemolytic anemia and/or thrombocytopenia 
Nephropathy that is related to WM 
Amyloidosis that is related to WM 
Hemoglobin ≤10 g/dL 
Platelet count <100 × 109/L 

Indications to start therapy are according to consensus criteria first published by Kyle et al31  and further confirmed in 201998  and in 2016.32 

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