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Chapter 54: Classification and Clinical Manifestations of the Malignant Lymphoid Disorders

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    AMA Citation
    Classification and Clinical Manifestations of the Malignant Lymphoid Disorders. In: Lichtman MA, Kaushansky K, Kipps TJ, Prchal JT, Levi MM. Lichtman M.A., Kaushansky K, Kipps T.J., Prchal J.T., Levi M.M. Eds. Marshall A. Lichtman, et al.eds. Williams Manual of Hematology, 8e New York, NY: McGraw-Hill; . http://hemonc.mhmedical.com/content.aspx?bookid=1783&sectionid=121722205. Accessed October 19, 2019.
    MLA Citation
    . "Classification and Clinical Manifestations of the Malignant Lymphoid Disorders." Williams Manual of Hematology, 8e Lichtman MA, Kaushansky K, Kipps TJ, Prchal JT, Levi MM. Lichtman M.A., Kaushansky K, Kipps T.J., Prchal J.T., Levi M.M. Eds. Marshall A. Lichtman, et al. New York, NY: McGraw-Hill, , http://hemonc.mhmedical.com/content.aspx?bookid=1783&sectionid=121722205.

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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 ...

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