Table 4

Recommendations for initial therapy of patients with WM, based on the individual patient characteristics

Clinical situation/individual patient characteristicsPrimary choice(s)Alternative (s)
Patients with WM-related cytopenias or organomegaly Rituximab-based combination Bortezomib/rituximab 
DRC 
Bendamustine/R 
Patients with symptomatic hyperviscosity, cryoglobulinemia or cold agglutinemia Bortezomib followed by bortezomib/rituximab Fludarabine/rituximab ± cyclophosphamide 
Bendamustine/rituximab 
Patients with paraprotein related neuropathy Rituximab alone Fludarabine/R 
DRC Bendamustine/rituximab 
Elderly patients with poor PS DRC Rituximab monotherapy 
Oral fludarabine Chlorambucil 
Elderly patients not eligible for systemic intravenous therapy Oral fludarabine Chlorambucil 
Young patients eligible for ASCT DRC Bendamustine/rituximab 
Bortezomib/rituximab R-CHOP 
Clinical situation/individual patient characteristicsPrimary choice(s)Alternative (s)
Patients with WM-related cytopenias or organomegaly Rituximab-based combination Bortezomib/rituximab 
DRC 
Bendamustine/R 
Patients with symptomatic hyperviscosity, cryoglobulinemia or cold agglutinemia Bortezomib followed by bortezomib/rituximab Fludarabine/rituximab ± cyclophosphamide 
Bendamustine/rituximab 
Patients with paraprotein related neuropathy Rituximab alone Fludarabine/R 
DRC Bendamustine/rituximab 
Elderly patients with poor PS DRC Rituximab monotherapy 
Oral fludarabine Chlorambucil 
Elderly patients not eligible for systemic intravenous therapy Oral fludarabine Chlorambucil 
Young patients eligible for ASCT DRC Bendamustine/rituximab 
Bortezomib/rituximab R-CHOP 
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