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Chapter 53: Classification and Clinical Manifestations of the Malignant Lymphoid Disorders

CLASSIFICATION

  • Lymphocyte malignancies comprise a wide spectrum of different morphologic and clinical syndromes.

  • The International Lymphoma Study Group proposed a new classification termed Revised European–American Lymphoma classification, or REAL classification, which was modified in 2001 and updated in 2008 by the World Health Organization (WHO) (see Table 53–1).

  • The REAL/WHO classification makes use of the pathologic, immunophenotypic, genetic, and clinical features to define separate disease entities (Table 53–1).

  • Lymphocytic neoplasms are divided into those derived from B cells, T cells, or natural killer (NK) cells.

  • The immunophenotype and the characteristic cytogenetic and genic abnormalities are also included in Table 53–1.

  • The most common indolent and aggressive lymphocytic neoplasms, based on their usual clinical behavior, are listed in Tables 53–2 and 53–3.

TABLE 53–1CLASSIFICATION OF LYMPHOMA AND LYMPHOID LEUKEMIA BY THE WORLD HEALTH ORGANIZATION
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TABLE 53–1CLASSIFICATION OF LYMPHOMA AND LYMPHOID LEUKEMIA BY THE WORLD HEALTH ORGANIZATION
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Neoplasm Morphology Phenotype* Genotype†
B-Cell Neoplasms
Immature B-Cell Neoplasms
Lymphoblastic leukemia/lymphoma not otherwise specified (NOS) (Chap. 54) Medium to large cells with finely stippled chromatin and scant cytoplasm TdT+, sIg-, CD10+, CD13+/-, CD19+, CD20-, CD22+, CD24+, CD34+/-, CD33+/-, CD45+/-, CD79a+, PAX5+ Clonal DJ rearrangement of IGH gene T(17;19), E2A-HLF, AML1 iAMP21 associated with poor prognosis
Lymphoblastic leukemia/lymphoma with recurrent genetic abnormalities (Chap. 54) See above See above. B-ALL with t(9;22) with CD25 and more frequent myeloid antigens CD13, CD33 See individual genetic features in B-ALL subtypes below
B-ALL with t(v;11q23); MLL rearranged See above CD19+, CD10-, CD24-, CD15+ Multiple MLL (11q23) fusion partners including AF4 (4q21), AF9 (9p22), and ENL (19p13). B-ALL with MLL translocations over express FLT-3. Poor prognosis
B-ALL with t(12;21) (p13;q22); TEL-AML1 (ETV6-RUNX1) See above CD19+, CD10+, CD34+. Characteristically negative for CD9, CD20, and CD66c t(12;21)(p13;q22) ETV6-RUNX translocation
B-ALL with hyperdiploidy See above CD19+, CD10+, CD45-, CD34+ Numerical increase in chromosomes without structural abnormalities. Most frequent chromosomes +21, X, 14 and 4. +1,2, 3 rarely seen. Favorable prognosis
B-ALL with hypodiploidy See above See above Loss of at least one or more chromosomes (range from 45 chromosomes to near haploid). Rare chromosome abnormalities. Poor prognosis
B-ALL with t(5;14) (q31;q32); IL3-IGH See above (with reactive eosinophilia) See above. Even rare blasts with B-ALL immunophenotype with eosinophilia strongly suggestive of this subtype of B-ALL t(5;14)(q31;q32); IL3-IGH leading to overexpression of IL3. Unclear prognosis
B-ALL with t(1;19) (q23;p13.3); E2A-PBX1 See above CD10+, CD19+, cytoplasmic μ heavy chain. CD9+, CD34- t(1;19)(q23;p13.3); leads to overexpression of E2A-PBX1 fusion gene product interfering with normal transcription factor activity of E2A and PBX1
Mature B-Cell Neoplasms
 Leukemias
 Chronic lymphocytic leukemia/small lymphocytic lymphoma (Chap. 55) Small cells with round, dense nuclei sIg+(dim), CD5+, CD10-, CD19+, CD20+(dim), CD22+(dim), CD23+, CD38+/-, CD45+, FMC-7- IgR+, trisomy 12 (~30%), del at 13q14 (~50%), 11q22–23, 17p13, and IGHV mutated status associated with poor prognosis

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