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1588 Part XI: Malignant Lymphoid Diseases Chapter 96: Pathology of Lymphomas 1589

attempts were made to incorporate these new entities into the existing WORLD HEALTH ORGANIZATION
classification schemes, problems with uniformity between different
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institutions persisted. A desire to eliminate the continued confusion CLASSIFICATION
ultimately led to a new approach to lymphoma classification proposed
by the International Lymphoma Study Group that used all available In the late 1990s, a new World Health Organization (WHO) classifica-
information, including morphology, immunophenotype, genetic and tion for lymphoproliferative disorders was being developed, based on
clinical features, to define a list of distinctive entities that could be uni- the REAL classification. First published in 2001 (and revised in 2008),
formly diagnosed by hematopathologists. The proposal was published the WHO classification represented a consensus between an interna-
in 1994 and was known as the Revised European-American Lymphoma tional group of more than 50 experienced hematopathologists, includ-
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(REAL) classification. Importantly, this classification identified enti- ing contributions from a clinical advisory committee of hematologists
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ties that had distinctive clinical features and could be reproducibly diag- and oncologists experienced in treating lymphomas. The WHO clas-
nosed by expert hematopathologists. 13 sification (Table 96–1) identified several major categories, including

TABLE 96–1. The WHO Classification of Lymphoid Neoplasms

PRECURSOR LYMPHOID NEOPLASMS • Intravascular large B-cell lymphoma
• B lymphoblastic leukemia/lymphoma, NOS • ALK-positive large B-cell lymphoma
• B lymphoblastic leukemia/lymphoma with recurrent genetic • Plasmablastic lymphoma
abnormalities • Large B-cell lymphoma arising in HHV-8–associated multicen-
• B lymphoblastic leukemia/lymphoma with t(9;22)(q34;q11.2); tric Castleman disease
BCR-ABL1 • Primary effusion lymphoma
• B lymphoblastic leukemia/lymphoma with t(v;11q23); MLL • Burkitt lymphoma
rearranged • B-cell lymphoma, unclassifiable, with features intermediate
• B lymphoblastic leukemia/lymphoma with t(12;21)(p13;q22); between DLBCL and Burkitt lymphoma
TEL-AML1 • B-cell lymphoma, unclassifiable, with features intermediate
• B lymphoblastic leukemia/lymphoma with hyperdiploidy between DLBCL and classical Hodgkin lymphoma
• B lymphoblastic leukemia/lymphoma with hypodiploidy MATURE T- AND NK-CELL NEOPLASMS
• B lymphoblastic leukemia/lymphoma with t(5;14)(q31;q32); • T-cell prolymphocytic leukemia
IL3-IGH • T-cell large granular lymphocytic leukemia
• B lymphoblastic leukemia/lymphoma with t(1;19)(q23;p13.3); • Chronic lymphoproliferative disorder of NK cells
E2A-PBX1
• T-lymphoblastic leukemia/lymphoma • Aggressive NK-cell leukemia
MATURE B-CELL NEOPLASMS • EBV-positive T-cell lymphoproliferative diseases of childhood
• Chronic lymphocytic leukemia/small lymphocytic lymphoma • Adult T-cell leukemia/lymphoma
• B-cell prolymphocytic leukemia • Extranodal NK/T-cell lymphoma, nasal type
• Splenic B-cell marginal zone lymphoma • Enteropathy-associated T-cell lymphoma
• Hairy cell leukemia • Hepatosplenic T-cell lymphoma
• Splenic B-cell lymphoma/leukemia, unclassifiable • Subcutaneous panniculitis-like T-cell lymphoma
• Lymphoplasmacytic lymphoma • Mycosis fungoides
• Heavy-chain diseases • Sézary syndrome
• Plasma cell neoplasms • Primary cutaneous CD30-positive T-cell lymphoproliferative
disorders
• Extranodal marginal zone lymphoma of mucosa-associated • Primary cutaneous peripheral T-cell lymphoma, rare subtypes
lymphoid tissue (MALT lymphoma)
• Nodal marginal zone lymphoma • Peripheral T-cell lymphoma, NOS
• Follicular lymphoma • Angioimmunoblastic T-cell lymphoma
• Primary cutaneous follicle center lymphoma • Anaplastic large cell lymphoma, ALK-positive
• Mantle cell lymphoma • Anaplastic large cell lymphoma, ALK-negative
• Diffuse large B-cell lymphoma (DLBCL), NOS HODGKIN LYMPHOMA
• T-cell/histiocyte rich large B-cell lymphoma • Nodular lymphocyte predominant Hodgkin lymphoma
• Primary DLBCL of the CNS • Classical Hodgkin lymphoma
• Primary cutaneous DLBCL, leg type • Nodular sclerosis classical Hodgkin lymphoma
• EBV-positive DLBCL of the elderly • Mixed cellularity classical Hodgkin lymphoma
• DLBCL associated with chronic inflammation • Lymphocyte-rich classical Hodgkin lymphoma
• Lymphomatoid granulomatosis • Lymphocyte-depleted classical Hodgkin lymphoma
• Primary mediastinal (thymic) large B-cell lymphoma

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