Pathologic Classification

Table 16-1 presents the World Health Organization (WHO) classification for NHL from the International Lymphoma Study Group. This classification for NHL is based on the currently recognized histologic (morphologic), immunophenotypic and genetic features, their clinical presentation and course. This updates the Revised European-American Lymphoma (REAL) classification and is the preferred classification for NHL.

Table 16-1. WHO Classification of Neoplastic Diseases of Hematopoietic and Lymphoid Tissues

Precursor B-cell neoplasms

Precursor B-lymphoblastic leukemia/lymphoma

Mature (peripheral) B-cell neoplasms

B-CLL/small lymphocytic lymphoma B-cell prolymphocytic leukemia Lymphoplasmacytic lymphoma Mantle-cell lymphoma Follicular lymphoma (grade 1, 2, or 3)

Nodal marginal zone B-cell lymphoma (+/- monocytoid B cells) Extranodal marginal zone B-cell lymphoma of MALT type Splenic marginal zone B-cell lymphoma (+/- villous lymphocytes) Hairy cell leukemia Plasma cell myeloma/plasmacytoma Diffuse large-B-cell lymphoma Morphologic variants

- Centroblastic

- Immunoblastic

- T-cell/histiocyte-rich

- Anaplastic large B-cell

- Plasmablastic Clinical variants

- Mediastinal (thymic) large B-cell lymphoma

- Primary effusion lymphoma

- Intravascular large B-cell lymphoma

- Lymphomatoid granulomatosis type Burkitt lymphoma/Burkitt cell leukemia

Morphologic variants

- Burkitt-like or typical Burkitt

- With plasmacytoid differentiation (AIDS-associated) Clinical and genetic variants

- Endemic

- Sporadic

- Immunodeficiency-associated

Table 16-1. (Continued)

Precursor T-cell neoplasms

Precursor T-lymphoblastic leukemia/lymphoma

Mature (peripheral) T-cell neoplasms

Predominantly leukemic/disseminated T-cell prolymphocytic leukemia T-cell granular lymphocytic leukemia Aggressive NK-cell leukemia Adult T-cell lymphoma/leukemia (HTLV1+) Predominantly nodal

Angioimmunoblastic T-cell lymphoma Peripheral T-cell lymphoma, not otherwise characterized Anaplastic large-cell lymphoma, T/null cell, primary systemic disease Predominantly extranodal

Mycosis fungoides/Sezary syndrome

Anaplastic large-cell lymphoma, T/null cell, primary cutaneous type Subcutaneous panniculitis-like T-cell lymphoma Extranodal NK/T-cell lymphoma, nasal type Enteropathy-type T-cell lymphoma Hepatosplenic gamma-delta T-cell lymphoma

Table 16-2 lists the histologic classification, relative frequency of histologic types, correlation with immunophenotype, common sites of involvement, and genetic features in childhood NHL. Fifty percent of NHL in children are small, noncleaved, 30% lymphoblastic, and 20% large-cell types.

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