Lymphoma classification


 * Associate Editor-in-Chief: Ross Bonander

WHO classification
The WHO Classification is the latest classification of lymphoma, published by the World Health Organization in 2001. It was based upon the "Revised European-American Lymphoma classification" (REAL).

This classification attempts to classify lymphomas by cell type, i.e. the normal cell type that most closely resembles the tumor. They are classified in three large groups: the B cell tumors, the T cell and natural killer cell tumors, Hodgkin lymphoma, and other minor groups: (ICD-O codes are provided where available)

Mature (peripheral) B-cell neoplasms

 * Chronic lymphocytic leukemia/small lymphocytic lymphoma
 * B-cell prolymphocytic leukemia
 * Lymphoplasmacytic lymphoma
 * Splenic marginal zone B-cell lymphoma (with or without villous lymphocytes)
 * Hairy cell leukemia
 * Splenic lymphoma/leukemia, unclassifiable
 * Plasma cell myeloma/plasmacytoma
 * Heavy chain diseases
 * Extranodal marginal zone B-cell lymphoma of MALT type (MALT lymphoma)
 * Nodal marginal zone B-cell lymphoma (with or without monocytoid B cells)
 * Follicular lymphoma
 * Primary cutaneous follicle center lymphoma
 * Mantle cell lymphoma
 * Diffuse large B-cell lymphoma (DLBCL)
 * Diffuse large B-cell lymphoma, not otherwise specified
 * T-cell /histiocyte rich large B-cell lymphoma
 * DLBCL associated with chronic inflammation
 * EBV positive DLBCL of the elderly
 * Lymphomatoid granulomatosis
 * Primary mediastinal (thymic) large B-cell lymphoma
 * Intravascular large B-cell lymphoma
 * Primary cutaneous DLBCL, leg type
 * ALK positive DLBCL
 * Plasmablastic lymphoma
 * Primary effusion lymphoma
 * Large B-cell lymphoma arising in HHV8-associated multicentric Castleman's disease
 * Burkitt lymphoma/Burkitt cell leukemia
 * B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma
 * B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma



Mature (peripheral) T/NK-cell neoplasms

 * T cell prolymphocytic leukemia
 * T cell large granular lymphocytic leukemia
 * Aggressive NK cell leukemia
 * Systemic EBV positive T-cell lymphoproliferative disease of childhood (associated with chronic active EBV infection)
 * Hydra vacciniforme-like lymphoma
 * Adult T-cell leukemia/lymphoma (ATLL)
 * Extranodal NK/T-cell lymphoma, nasal type
 * Hepatosplenic T-cell lymphoma
 * Subcutaneous pannicultis-like T-cell lymphoma
 * Mycosis fungoides / Sezary syndrome
 * Primary cutaneous anaplastic large cell lymphoma
 * Primary cutaneous aggressive epidermotropic CD8 positive cytotoxic T-cell lymphoma
 * Primary cutaneous gamma-delta T-cell lymphoma
 * Primary cutaneous small/medium CD4 positive T-cell lymphoma
 * Peripheral T cell lymphoma, NOS (not otherwise specified)
 * Angioimmunoblastic T-cell lymphoma
 * Anaplastic large cell lymphoma, ALK positive
 * Anaplastic large cell lymphoma, ALK negative

Hodgkin Lymphoma

 * Nodular lymphocyte-predominant Hodgkin lymphoma
 * Classical Hodgkin lymphoma
 * Nodular sclerosis
 * Mixed cellularity
 * Lymphocyte-rich
 * Lymphocyte depleted or not depleted

Immunodeficiency-associated lymphoproliferative disorders

 * Associated with a primary immune disorder
 * Associated with the Human Immunodeficiency Virus (HIV)
 * Post-transplant
 * Associated with Methotrexate therapy

Working formulation
The Working Formulation, published in 1982, is primarily descriptive. It is still occasionally used, but has been superseded by the WHO classification, above.

Low grade

 * Malignant Lymphoma, small lymphocytic (chronic lymphocytic leukemia)
 * Malignant Lymphoma, follicular, predominantly small cleaved cell
 * Malignant Lymphoma, follicular, mixed (small cleaved and large cell)

High grade

 * Malignant Lymphoma, large cell, immunoblastic
 * Malignant Lymphoma, lymphoblastic
 * Malignant Lymphoma, small non-cleaved cells (Burkitt's lymphoma)

Miscellaneous

 * Composite
 * Histiocytic
 * Extramedullary plasmacytoma
 * Unclassifiable

Other classification systems

 * ICD-O (codes 9590-9999, details at )
 * ICD-10 (codes C81-C96, details at )