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Pathology (WHO 2016)

Mature B-cell neoplasms

  • Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma

  • Monoclonal B-cell lymphocytosis*

  • B-cell prolymphocytic leukemia

  • Splenic marginal zone lymphoma

  • Hairy cell leukemia

  • Splenic B-cell lymphoma/leukemia, unclassifiable

    • Splenic diffuse red pulp small B-cell lymphoma

    • Hairy cell leukemia-variant

  • Lymphoplasmacytic lymphoma

    • Waldenström macroglobulinemia

  • Monoclonal gammopathy of undetermined significance (MGUS), IgM*

  • μ heavy-chain disease

  • γ heavy-chain disease

  • α heavy-chain disease

  • Monoclonal gammopathy of undetermined significance (MGUS), IgG/A*

  • Plasma cell myeloma

  • Solitary plasmacytoma of bone

  • Extraosseous plasmacytoma

  • Monoclonal immunoglobulin deposition diseases*

  • Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)

  • Nodal marginal zone lymphoma

    • Pediatric nodal marginal zone lymphoma

  • Follicular lymphoma

    • In situ follicular neoplasia*

    • Duodenal-type follicular lymphoma*

  • Pediatric-type follicular lymphoma*

  • Large B-cell lymphoma with IRF4 rearrangement*†

  • Primary cutaneous follicle center lymphoma

  • Mantle cell lymphoma

    • In situ mantle cell neoplasia*

  • Diffuse large B-cell lymphoma (DLBCL), NOS

    • Germinal center B-cell type*

    • Activated B-cell type*

  • T-cell/histiocyte-rich large B-cell lymphoma

  • Primary DLBCL of the central nervous system (CNS)

  • Primary cutaneous DLBCL, leg type


  • EBV+ mucocutaneous ulcer*†

  • DLBCL associated with chronic inflammation

  • Lymphomatoid granulomatosis

  • Primary mediastinal (thymic) large B-cell lymphoma

  • Intravascular large B-cell lymphoma

  • ALK+ large B-cell lymphoma

  • Plasmablastic lymphoma

  • Primary effusion lymphoma

  • HHV8+ DLBCL, NOS*†

  • Burkitt lymphoma

  • Burkitt-like lymphoma with 11q aberration*†

  • High-grade B-cell lymphoma, with MYC and BCL2 and/or BCL6 rearrangements*

  • High-grade B-cell lymphoma, NOS*

  • B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and classic Hodgkin lymphoma

Mature T And NK Neoplasms

  • T-cell prolymphocytic leukemia

  • T-cell large granular lymphocytic leukemia

  • Chronic lymphoproliferative disorder of NK cells

  • Aggressive NK-cell leukemia

  • Systemic EBV+ T-cell lymphoma of childhood*

  • Hydroa vacciniforme–like lymphoproliferative disorder*

  • Adult T-cell leukemia/lymphoma

  • Extranodal NK-/T-cell lymphoma, nasal type

  • Enteropathy-associated T-cell lymphoma

  • Monomorphic epitheliotropic intestinal T-cell lymphoma*

  • Indolent T-cell lymphoproliferative disorder of the GI tract*†

  • Hepatosplenic T-cell lymphoma

  • Subcutaneous panniculitis-like T-cell lymphoma

  • Mycosis fungoides

  • Sézary syndrome

  • Primary cutaneous CD30+ T-cell lymphoproliferative disorders

    • Lymphomatoid papulosis

    • Primary cutaneous anaplastic large cell lymphoma

  • Primary cutaneous γδ T-cell lymphoma

  • Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma

  • Primary cutaneous acral CD8+ T-cell lymphoma*†

  • Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder*†

  • Peripheral T-cell lymphoma, NOS

  • Angioimmunoblastic T-cell lymphoma

  • Follicular T-cell lymphoma*†

  • Nodal peripheral T-cell lymphoma with TFH phenotype*†

  • Anaplastic large-cell lymphoma, ALK+

  • Anaplastic large-cell lymphoma, ALK-*

  • Breast implant–associated anaplastic large-cell lymphoma*†

Posttransplant Lymphoproliferative Disorders (PTLD)

  • Plasmacytic hyperplasia PTLD

  • Infectious mononucleosis PTLD

  • Florid follicular hyperplasia PTLD*

  • Polymorphic PTLD

  • Monomorphic PTLD (B- and T-/NK-cell types)

  • Classic Hodgkin lymphoma PTLD

Histiocytic and Dendritic Cell Neoplasms

  • Histiocytic sarcoma

  • Langerhans cell histiocytosis

  • Langerhans cell sarcoma

  • Indeterminate dendritic cell tumor

  • Interdigitating dendritic cell sarcoma

  • Follicular dendritic cell sarcoma

  • Fibroblastic reticular cell tumor

  • Disseminated juvenile xanthogranuloma

  • Erdheim-Chester disease*


*Changes from the WHO 2008 classification

Provisional entity

Swerdlow SH et al, eds. World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th Edition. Lyon, France: IARC ...

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