Table 3.

Frontline therapeutic options for Waldenström macroglobulinemia


Therapeutic class and agents*

Evidence for efficacy21 

Level of recommendation21 
Alkylator agents   
    Chlorambucil   IIa   B  
Nucleoside analogs   
    Cladribine or fludarabine   IIa   B  
Monoclonal antibody   
    Rituximab (standard or extended schedule)   IIa   B  
Nucleoside analogs plus alkylators   
    Cladribine or fludarabine plus cyclophosphamide   IIa   B  
Nucleoside analogs plus rituximab   
    Fludarabine plus rituximab   IIa   B  
Nucleoside analogs plus alkylators and rituximab   
    Cladribine, cyclophosphamide, and rituximab   IIa   B  
    Fludarabine, cyclophosphamide, and rituximab   III   C  
    Pentostatin, cyclophosphamide, and rituximab   III   C  
Combination chemotherapy plus rituximab   
    CHOP and rituximab   IIa   B  
    Cyclophosphamide, dexamethasone, and rituximab
 
IIa
 
B
 

Therapeutic class and agents*

Evidence for efficacy21 

Level of recommendation21 
Alkylator agents   
    Chlorambucil   IIa   B  
Nucleoside analogs   
    Cladribine or fludarabine   IIa   B  
Monoclonal antibody   
    Rituximab (standard or extended schedule)   IIa   B  
Nucleoside analogs plus alkylators   
    Cladribine or fludarabine plus cyclophosphamide   IIa   B  
Nucleoside analogs plus rituximab   
    Fludarabine plus rituximab   IIa   B  
Nucleoside analogs plus alkylators and rituximab   
    Cladribine, cyclophosphamide, and rituximab   IIa   B  
    Fludarabine, cyclophosphamide, and rituximab   III   C  
    Pentostatin, cyclophosphamide, and rituximab   III   C  
Combination chemotherapy plus rituximab   
    CHOP and rituximab   IIa   B  
    Cyclophosphamide, dexamethasone, and rituximab
 
IIa
 
B
 

Information has been updated from the original consensus panel recommendations of the Second International Workshop on Waldenström's Macroglobulinemia.

*

The choice of appropriate therapy should take into account the candidacy of a patient for high-dose chemotherapy since prolonged use of both alkylating agents and nucleoside analogs can deplete hematopoietic stem cells.

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