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 |Favorite Table|Download (.pdf) TABLE 54–1 INDOLENT LYMPHOMAS
Chronic lymphocytic leukemia
Hairy cell leukemia
Splenic marginal zone B-cell lymphoma (with or without villous lymphocytes)
Plasma cell myeloma/plasmacytoma
Nodal marginal zone B-cell lymphoma (with or without monocytoid B cells)
Small lymphocytic lymphoma
Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type
TABLE 54–2AGGRESSIVE LYMPHOMAS |Favorite Table|Download (.pdf) TABLE 54–2 AGGRESSIVE LYMPHOMAS
Immature B-Cell Neoplasms
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
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
TABLE 54–3CLASSIFICATION OF LYMPHOMA AND LYMPHOID LEUKEMIA BY WORLD HEALTH ORGANIZATION |Favorite Table|Download (.pdf) 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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