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Waldenström’s macroglobulinemia – a review
Rev Assoc Med B ras 2014; 60(5):490-499 495
Table 3Differential diagnosis of Waldenström’s macroglobulinemia (WM) (Adapted from Fonseca and Hayman 200776)
Neoplasm Immunophenotype Pattern of marrow infiltration and cell
morphology
Cytogenetic abnormalities
Waldenström’s
Macroglobulinemia
sIgM+(kappa to lambda ratio 5:1),
sIgD+ , CD19+, CD20+, CD22+,
CD79a+, PAX5+, Bcl2+, FMC7+,
CD5+/-, Bcl6-, CD10- , CD23-,
CD25+ CD27+, CD103- , CD138-,
CD56-
Generally intertrabecular;
Small lymphocytes with plasmacytoid
differentiation
Most patients have a normal karyotype;
The most frequent cytogenetic alterations are
6q21-23 deletions
Multiple Myeloma cIg+, sIg-, CD19- , CD20-, PAX5-,
CD38+, CD79a+, CD138+, CD56+
Nodular, diffuse, interstitial;
Plasma cells with different degrees of
maturation
t(11;14) (q13;q32)
B-cell chronic
lymphocytic
leukemia
sIg + (weak), CD43 + (weak),
CD20+ (weak), CD19+, CD23+,
CD5+ , CD23+, CD10-, CD79b/
CD22-, FMC7 -, Cyclin D1-
Nodular, interstitial, diffuse or mix of
all three;
Small lymphocytes with dense nucleus,
aggregated chromatin, no visible nucleoli
and reduced cytoplasm
Del 13q14 (50% of cases);
Del 11q (20% of cases);
Trisomy of chromosome 12 (20% of cases);
Del 17p;
Presence of ZAP-70 + (Tyrosine Kinase of 70
Kda associated to the zeta chain of the T
lymphocyte receptor complex)
Mantle cell
lymphoma
sIgM+ ,sIgD+/-, lambda light chain
restriction, CD19+, CD20+, CD5+,
CD43+, FMC-7+, Cyclin D1+,
CD10-, BCL-6-, CD23 -
Variable infiltration pattern.
Lymphocytes are small or medium in
size with irregular nucleus
t(11;14) (q13;q32)
Follicular
lymphoma
sIg +, CD10+, CD19 +, CD20+,
CD21+, CD22 +, CD79a+, Bcl-
2+ ,Bcl-6+, CD43-, CD5-, CD23-,
CD43-
Paratrabecular infiltration;
Centrocytes (small cells with “cleaved”
nuclei and reduced cytoplasm) and
centroblasts (large cells with round or
oval nuclei, vesicular chromatin, and
low basophilic cytoplasm)
t(14;18)(q32;q21) (70-95% of cases).
Extranodal
marginal zone
lymphoma, MALT
sIgM+ (generally), light chain
restriction (generally), CD19+,
CD20+, CD21+, CD35+, CD22+,
CD79+, CD43+/-, CD5- , CD10-,
CD23-, CD11c+ (weak)/CD11c-
Variable Trisomy 3 (60% of cases);
t(11;18) (q21;q21) (25%-50% of cases);
t(1;14) (p22;q32);
t(11;18) (q21;q22);
t(14;18) (q32;q21);
t(3;14) (p13;q32)
Nodal marginal
zone lymphoma
Most lymphomas have a similar
immunophenotype to MALT
lymphoma, others have a similar
immunophenotype to splenic
marginal zone lymphomas
Variable Trisomy 3;
t(11;18) (q21;q21)
Splenic marginal
zone lymphoma
sIgM+, IgD+(generally), CD19+,
CD20+ , CD22+, Bcl-2+, CD79a+,
CD5- , CD23-, CD10- , CD43-,
CD25-, CD103-, Cyclin D1-
Nodular, interstitial Chromosomal gains: 3q (30-40% of cases), 5q
(28%), 12q (24%), 20q (24%), 9q (21%), 4q
(17%);
Trisomy 3 (17%);
Del 7q;
Del 6q and Del 17p – Genetic alterations
associated with clinical progression of the
disease
cIg – cytoplasmic immunoglobulin, sIg, surface immunoglobulin; MALT, mucosa-associated lymphoid tissue.