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Differential diagnosis of Waldenstrom macroglobulinemia

Differential diagnosis of Waldenstrom macroglobulinemia
Entity Bone marrow biopsy Monoclonal gammopathy Additional features
Waldenstrom macroglobulinemia ≥10 percent infiltration by lymphoplasmacytic lymphoma Circulating monoclonal IgM of any size Anemia, hepatosplenomegaly, lymphadenopathy, and/or systemic symptoms
Smoldering Waldenstrom macroglobulinemia

Circulating monoclonal IgM ≥3.0 g/dL

and/or

≥10 percent infiltration by lymphoplasmacytic lymphoma

No anemia, hepatosplenomegaly, lymphadenopathy, or systemic symptoms felt to be related to underlying lymphoplasmacytic proliferation
IgM MGUS Minimal or no lymphoplasmacytic infiltration of the bone marrow; overall marrow involvement by such cells should be <10 percent Circulating monoclonal IgM concentration <3.0 g/dL No anemia, hepatosplenomegaly, lymphadenopathy, or systemic symptoms felt to be related to underlying lymphoplasmacytic proliferation
Multiple myeloma Infiltration of plasma cells in the bone marrow IgM myeloma is rare; more commonly monoclonal protein is IgG, IgA, light chains only, or infrequently IgD Hypercalcemia, renal insufficiency, anemia, and bone lesions felt to be related to underlying lymphocyte proliferative disorder
Chronic lymphocytic leukemia/small lymphocytic lymphoma Bone marrow infiltration with small mature appearing lymphocytes that express CD5; expression of CD20 and surface Ig is dim Monoclonal immunoglobulins can be seen in up to 15 percent Anemia, lymphadenopathy, hepatosplenomegaly, and/or systemic symptoms felt to be related to underlying plasma cell proliferative disorder
Mantle cell lymphoma Bone marrow infiltration with monomorphous small to medium-sized B lymphocytes that overexpress cyclin D1 IgM monoclonal protein may be present t(11;14)
Amyloidosis Apple green birefringence under polarized light after staining with Congo red Usually have an associated monoclonal protein Nephrotic syndrome, restrictive cardiomyopathy, peripheral neuropathy, hepatomegaly, macroglossia, and purpura
MGUS: monoclonal gammopathy of undetermined significance.
Graphic 82116 Version 2.0

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