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1494 Part XI: Malignant Lymphoid Diseases Chapter 90: Classification of Malignant Lymphoid Disorders 1495

TABLE 90–1. Classification of Lymphoma and Lymphoid Leukemia by World Health Organization (Continued)
Neoplasm Morphology Phenotype* Genotype †
Follicular lymphoma Small, medium, or large sIg, CD5-, CD10+, CD19+, IgR, t(14;18)(q32;q21) (~85%) involv-
(follicle center lym- cells with cleaved nuclei CD20+(bright), CD23-/+, CD38+, ing BCL2 and IgH. Mutated 3q27
phoma; Chap. 99) CD45+ (5-15%, BCL6)
Nodal marginal zone Small or large monocytoid sIgM+, sIgD-, cIg+ (~50%), CD5-, IgR, commonly with trisomies 3, 7,
B-cell lymphoma cells CD10-, CD11c+/-, CD19+, CD20+, and 18
(Chap. 101) CD23-, CD43+/-
Extranodal marginal See above See above t(11;18)(q21;q21) involving API2,
zone lymphoma of MLT1, or t(1;14)(p22;q32) involving
mucosa-associated BCL10
lymphoid tissue (MALT)
type (Chap. 101)
Splenic B-cell marginal Small round lymphocytes sIgM+, sIgD-, CD5+/-, CD19+, CD20+, IgR, allelic loss of chromosome 7q31–
zone lymphoma replaces reactive germinal CD23-, CD103- 32 (40%)
centers and/or villous lym-
phocytes in blood
Splenic B-cell lymphoma, unclassifiable
Splenic diffuse red pulp Blood: villous lymphocytes CD20+, DBA.44+, IgG+/IgD-, CD25-, T(9;14)(p13;q32) involving PAX5 and
small B-cell lymphoma similar to SMZL. Marrow: CD5-, CD103-, CD123- IGH
intrasinusoidal infiltration.
Spleen: monomorphous
small to medium lympho-
cytes with round nuclei,
vesicular chromatin, occa-
sional small nucleoli
Hairy cell leukemia Hybrid features of prolym- sIg+(bright), CD5-, CD10-, BRAF mutation negative
variant phocytic leukemia and clas- CD11c+(bright), CD19+, CD20+,
sic hairy cell leukemia CD25-, CD45+, CD103+, FMC7+,
CD123-, Annexin A1-, TRAP–
Diffuse large B-cell lymphoma (DLBCL, Chap. 98)
DLBCL NOS
Common Morphologic Variants:
Centroblastic Medium to large lymphoid sIgM+, sIgD+/-, CD5-, CD10-/+, IgR, 3q27 abnormalities and/or t(3;14)
cells with vesicular nuclei CD19+, CD20+, CD22+, CD79a+, (q27;q32) involving BCL6 (~30%) or
containing fine chromatin. CD45+, PAX5+ t(14;18)(q32;q21) (~25%) involving
Multiple nucleoli BCL2
Immunoblastic >90% of cells are immu- See above. May express CD30+ See above
noblasts with central
nucleolus
Anaplastic Very large round, oval, or See above. Often CD30+ See above
polygonal cells with bizarre
pleomorphic nuclei resem-
bling R-S cells
Molecular Subgroups
Germinal center B cell See above See above See above
like (GCB)
Activated B-cell–like See above. Often with See above T(14;18) (35%), 12q12 (20%), IG muta-
(ABC) more immunoblastic tion, BCL2 rearrangement (20–25%),
morphology Rel amplification (15%). Amplification
of microRNA-17-92 cluster
Immunohistochemical Gain of 3q (26%), 9p (6%), 12q12 (5%),
Subgroups NF-κB activation
CD5-positive DLBCL See above See above. CD5+ t(11;14) and t(14;18) negative. +3 and
gain on chromosome 16/16p and
18/18q common. Deletion p16/INK4a
(Continued)

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