Table 11.

Classification of Waldenström’s macroglobulinemia (WM) and related disorders.

DisorderLaboratory FeaturesClinical Features
a sIg+CD19+CD20+CD22+CD79+ 
b Chronic lymphocytic leukemia (CLL), diffuse large B-cell lymphoma, extranodal marginal-zone lymphoma, follicular lymphoma and mantle-cell lymphoma. 
Abbreviations: HCD, heavy-chain disease; MGUS, monoclonal gammopathy of undetermined significance. 
WM Lymphoplasmacytic infiltrate in marrow 
 Specific immunophenotypea
 Serum monoclonal IgM May be symptomatic or asymptomatic 
IgM-related disorder No marrow infiltration Symptomatic, e.g., peripheral neuropathy,
 cryoglobulins, cold agglutinin disease, AL amyloidosis 
IgM MGUS No marrow infiltrate Asymptomatic 
Other B cell 
 lymphoproliferative 
 disordersb Differentiate by immunophenotype and
 morphological characteristics  
μ-HCD Heavy-chain fragment with no associated
 light chain  
IgM myeloma IgM-producing plasma cells (cytoplasmic 
 IgM+, CD20+ CD138+, t(11;14)) Possibly associated with lytic skeletal lesions and
 hypercalcemia 
DisorderLaboratory FeaturesClinical Features
a sIg+CD19+CD20+CD22+CD79+ 
b Chronic lymphocytic leukemia (CLL), diffuse large B-cell lymphoma, extranodal marginal-zone lymphoma, follicular lymphoma and mantle-cell lymphoma. 
Abbreviations: HCD, heavy-chain disease; MGUS, monoclonal gammopathy of undetermined significance. 
WM Lymphoplasmacytic infiltrate in marrow 
 Specific immunophenotypea
 Serum monoclonal IgM May be symptomatic or asymptomatic 
IgM-related disorder No marrow infiltration Symptomatic, e.g., peripheral neuropathy,
 cryoglobulins, cold agglutinin disease, AL amyloidosis 
IgM MGUS No marrow infiltrate Asymptomatic 
Other B cell 
 lymphoproliferative 
 disordersb Differentiate by immunophenotype and
 morphological characteristics  
μ-HCD Heavy-chain fragment with no associated
 light chain  
IgM myeloma IgM-producing plasma cells (cytoplasmic 
 IgM+, CD20+ CD138+, t(11;14)) Possibly associated with lytic skeletal lesions and
 hypercalcemia 
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