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Chapter 54: 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 54–1).

  • The REAL/WHO classification makes use of the pathologic, immunophenotypic, genetic, and clinical features to define separate disease entities (Table 54–1).

  • Distinctive cytogenetic abnormalities are also described in Table 54–1.

  • Tumors are divided into two categories: indolent versus aggressive (Tables 54–2 and 54–3).

TABLE 54–1INDOLENT LYMPHOMAS
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TABLE 54–1 INDOLENT LYMPHOMAS

Disseminated Lymphomas/Leukemias

 Chronic lymphocytic leukemia

 Hairy cell leukemia

 Lymphoplasmacytic lymphoma

 Splenic marginal zone B-cell lymphoma (with or without villous lymphocytes)

 Plasma cell myeloma/plasmacytoma

Nodal Lymphomas

 Follicular lymphoma

 Nodal marginal zone B-cell lymphoma (with or without monocytoid B cells)

 Small lymphocytic lymphoma

 Extranodal lymphomas

 Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type

Source: Williams Hematology, 8th ed, Chap. 92, Table 92–2, p. 1407.

TABLE 54–2AGGRESSIVE LYMPHOMAS
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TABLE 54–2 AGGRESSIVE LYMPHOMAS

Immature B-Cell Neoplasms

 B-lymphoblastic leukemia/lymphoma

Mature B-Cell Neoplasms

 Burkitt lymphoma/Burkitt cell leukemia

 Diffuse large B-cell lymphoma

 Follicular lymphoma grade III

 Mantle cell lymphoma

Immature T-Cell Neoplasms

 T-lymphoblastic lymphoma/leukemia

Peripheral T- and Natural Killer (NK) Cell Neoplasms

 T-cell prolymphocytic leukemia/lymphoma

 Aggressive NK cell leukemia/lymphoma

 Adult T-cell lymphoma/leukemia (associated with HTLV-1 [human T-cell leukemia virus type 1])

 Extranodal NK/T cell lymphoma

 Enteropathy-associated T-cell lymphoma

 Hepatosplenic T-cell lymphoma

 Subcutaneous panniculitis-like T-cell lymphoma

 Peripheral T-cell lymphomas, not otherwise specified

 Angioimmunoblastic T-cell lymphoma

 Anaplastic large cell lymphoma, primary, systemic

Source: Williams Hematology, 8th ed, Chap. 92, Table 92–3, p. 1407.

TABLE 54–3CLASSIFICATION OF LYMPHOMA AND LYMPHOID LEUKEMIA BY WORLD HEALTH ORGANIZATION
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TABLE 54–3 CLASSIFICATION OF LYMPHOMA AND LYMPHOID LEUKEMIA BY WORLD HEALTH ORGANIZATION
Neoplasm Morphology Phenotype Genotype
B-Cell Neoplasms
Immature B-Cell Neoplasms
Lymphoblastic leukemia (see Chap. 55) Medium to large cells with finely stippled chromatin and scant cytoplasm TdT+, sIg-, CD10+, CD13+/-, CD19+, CD20-, CD22+, CD34+/-, CD33+/-, CD45+/-, CD79a+ t(1;19), t(9;22), and defects at 11q23-defects associated with poor prognosis
Lymphoblastic lymphoma (see Chap. 65) Medium-sized cells with high nuclear to cytoplasmic ratio See above See above
Mature B-cell Neoplasms
Leukemias
Chronic lymphocytic leukemia (see Chap. 56) 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%), 11q- (15%)
Prolymphocytic leukemia (see Chap. 56) ≥55% prolymphocytes sIg+(bright), CD5+/-, CD10-, CD19+, CD22+, CD23+/-, CD45+ IgR, trisomy 12 (~30%)
Hairy cell leukemia (see Chap. 57) Small cells with cytoplasmic projections sIg+(bright), CD5-, CD10-, CD11c+(bright), CD19+, CD20+, CD25+, CD45+, CD103+, Annexin A+ IgR,
Lymphomas
Small lymphocytic lymphoma (see Chap. 56) Small round ...

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