Table 1.

International Consensus Classification of mature lymphoid and histiocytic/dendritic cell neoplasms

Mature B-cell neoplasms 
 Chronic lymphocytic leukemia/small lymphocytic lymphoma 
 Monoclonal B-cell lymphocytosis 
  Chronic lymphocytic leukemia type 
  Non-chronic lymphocytic leukemia type 
 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 
 Immunoglobulin M (IgM) monoclonal gammopathy of undetermined significance (MGUS) 
  IgM MGUS, plasma cell type* 
  IgM MGUS, not otherwise specified (NOS)* 
 Primary cold agglutinin disease* 
 Heavy chain diseases 
  Mu heavy chain disease 
  Gamma heavy chain disease 
  Alpha heavy chain disease 
 Plasma cell neoplasms 
  Non-IgM MGUS 
  Multiple myeloma (plasma cell myeloma)* 
   Multiple myeloma, NOS 
   Multiple myeloma with recurrent genetic abnormality 
    Multiple myeloma with CCND family translocation 
    Multiple myeloma with MAF family translocation 
    Multiple myeloma with NSD2 translocation 
    Multiple myeloma with hyperdiploidy 
  Solitary plasmacytoma of bone 
  Extraosseous plasmacytoma 
 Monoclonal Ig deposition diseases 
  Ig light chain amyloidosis (AL)* 
  Localized AL amyloidosis* 
  Light chain and heavy chain deposition disease 
 Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) 
 Primary cutaneous marginal zone lymphoproliferative disorder* 
 Nodal marginal zone lymphoma 
  Pediatric nodal marginal zone lymphoma 
 Follicular lymphoma 
  In situ follicular neoplasia 
  Duodenal-type follicular lymphoma 
 BCL2-R–negative, CD23-positive follicle center lymphoma 
 Primary cutaneous follicle center lymphoma 
 Pediatric-type follicular lymphoma 
 Testicular follicular lymphoma* 
 Large B-cell lymphoma with IRF4 rearrangement* 
 Mantle cell lymphoma 
  In situ mantle cell neoplasia 
  Leukemic non-nodal mantle cell lymphoma 
 Diffuse large B-cell lymphoma, NOS 
  Germinal center B-cell subtype 
  Activated B-cell subtype 
 Large B-cell lymphoma with 11q aberration* 
 Nodular lymphocyte predominant B-cell lymphoma* 
 T cell/histiocyte-rich large B-cell lymphoma 
 Primary diffuse large B-cell lymphoma of the central nervous system 
 Primary diffuse large B-cell lymphoma of the testis* 
 Primary cutaneous diffuse large B-cell lymphoma, leg type 
 Intravascular large B-cell lymphoma 
 HHV-8 and Epstein-Barr virus–negative primary effusion-based lymphoma* 
 Epstein-Barr virus–positive mucocutaneous ulcer* 
 Epstein-Barr virus–positive diffuse large B-cell lymphoma, NOS 
 Diffuse large B-cell lymphoma associated with chronic inflammation 
  Fibrin-associated diffuse large B-cell lymphoma 
 Lymphomatoid granulomatosis 
 Epstein-Barr virus–positive polymorphic B-cell lymphoproliferative disorder, NOS* 
 ALK-positive large B-cell lymphoma 
 Plasmablastic lymphoma 
 HHV-8–associated lymphoproliferative disorders 
  Multicentric Castleman disease 
  HHV-8–positive germinotropic lymphoproliferative disorder 
  HHV-8–positive diffuse large B-cell lymphoma, NOS 
  Primary effusion lymphoma 
 Burkitt lymphoma 
 High-grade B-cell lymphoma, with MYC and BCL2 rearrangements* 
 High-grade B-cell lymphoma with MYC and BCL6 rearrangements* 
 High-grade B-cell lymphoma, NOS 
 Primary mediastinal large B-cell lymphoma 
 Mediastinal gray-zone lymphoma* 
Classic Hodgkin lymphoma 
 Nodular sclerosis classic Hodgkin lymphoma 
 Lymphocyte-rich classic Hodgkin lymphoma 
 Mixed cellularity classic Hodgkin lymphoma 
 Lymphocyte-depleted classic Hodgkin lymphoma 
Mature T-cell and NK-cell neoplasms 
 T-cell prolymphocytic leukemia 
 T-cell large granular lymphocytic leukemia 
 Chronic lymphoproliferative disorder of NK cells 
 Adult T-cell leukemia/lymphoma 
 Epstein-Barr virus–positive T-cell/NK-cell lymphoproliferative disorders of childhood* 
  Hydroa vacciniforme lymphoproliferative disorder 
   Classic 
   Systemic 
  Severe mosquito bite allergy 
  Chronic active Epstein-Barr virus disease, systemic (T-cell and NK-cell phenotype) 
  Systemic Epstein-Barr virus–positive T-cell lymphoma of childhood 
 Extranodal NK/T-cell lymphoma, nasal type 
 Aggressive NK-cell leukemia 
 Primary nodal Epstein-Barr virus–positive T-cell/NK-cell lymphoma* 
 Enteropathy-associated T-cell lymphoma 
  Type II refractory celiac disease* 
 Monomorphic epitheliotropic intestinal T-cell lymphoma 
 Intestinal T-cell lymphoma, NOS 
 Indolent clonal T-cell lymphoproliferative disorder of the gastrointestinal tract* 
 Indolent NK-cell lymphoproliferative disorder of the gastrointestinal tract* 
 Hepatosplenic T-cell lymphoma 
 Mycosis fungoides 
 Sézary syndrome 
 Primary cutaneous CD30+ T-cell lymphoproliferative disorders 
  Lymphomatoid papulosis 
  Primary cutaneous anaplastic large cell lymphoma 
 Primary cutaneous small/medium CD4+ T-cell lymphoproliferative disorder 
 Subcutaneous panniculitis-like T-cell lymphoma 
 Primary cutaneous gamma-delta T-cell lymphoma 
 Primary cutaneous acral CD8+ T-cell lymphoproliferative disorder* 
 Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma 
 Peripheral T-cell lymphoma, NOS 
 Follicular helper T-cell lymphoma* 
  Follicular helper T-cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma) 
  Follicular helper T-cell lymphoma, follicular type 
  Follicular helper T-cell lymphoma, NOS 
 Anaplastic large cell lymphoma, ALK positive 
 Anaplastic large cell lymphoma, ALK negative 
 Breast implant–associated anaplastic large cell lymphoma 
Immunodeficiency-associated lymphoproliferative disorders 
 Posttransplant lymphoproliferative disorders 
  Nondestructive posttransplant lymphoproliferative disorders 
   Plasmacytic hyperplasia posttransplant lymphoproliferative disorder 
   Infectious mononucleosis posttransplant lymphoproliferative disorder 
   Florid follicular hyperplasia posttransplant lymphoproliferative disorder 
  Polymorphic posttransplant lymphoproliferative disorder 
  Monomorphic posttransplant lymphoproliferative disorder (B-cell and T-cell/NK-cell types) 
  Classic Hodgkin lymphoma posttransplant lymphoproliferative disorder 
 Other iatrogenic immunodeficiency-associated lymphoproliferative disorders 
Histiocytic and dendritic cell neoplasms 
 Histiocytic sarcoma 
 Langerhans cell histiocytosis 
 Langerhans cell sarcoma 
 Indeterminate dendritic cell histiocytosis* 
 Interdigitating dendritic cell sarcoma* 
 ALK-positive histiocytosis* 
 Disseminated juvenile xanthogranuloma 
 Erdheim-Chester disease 
 Rosai-Dorfman-Destombes disease* 
 Follicular dendritic cell sarcoma 
 Fibroblastic reticular cell sarcoma* 
 Epstein-Barr virus–positive inflammatory follicular dendritic cell/fibroblastic reticular cell tumor* 
Mature B-cell neoplasms 
 Chronic lymphocytic leukemia/small lymphocytic lymphoma 
 Monoclonal B-cell lymphocytosis 
  Chronic lymphocytic leukemia type 
  Non-chronic lymphocytic leukemia type 
 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 
 Immunoglobulin M (IgM) monoclonal gammopathy of undetermined significance (MGUS) 
  IgM MGUS, plasma cell type* 
  IgM MGUS, not otherwise specified (NOS)* 
 Primary cold agglutinin disease* 
 Heavy chain diseases 
  Mu heavy chain disease 
  Gamma heavy chain disease 
  Alpha heavy chain disease 
 Plasma cell neoplasms 
  Non-IgM MGUS 
  Multiple myeloma (plasma cell myeloma)* 
   Multiple myeloma, NOS 
   Multiple myeloma with recurrent genetic abnormality 
    Multiple myeloma with CCND family translocation 
    Multiple myeloma with MAF family translocation 
    Multiple myeloma with NSD2 translocation 
    Multiple myeloma with hyperdiploidy 
  Solitary plasmacytoma of bone 
  Extraosseous plasmacytoma 
 Monoclonal Ig deposition diseases 
  Ig light chain amyloidosis (AL)* 
  Localized AL amyloidosis* 
  Light chain and heavy chain deposition disease 
 Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) 
 Primary cutaneous marginal zone lymphoproliferative disorder* 
 Nodal marginal zone lymphoma 
  Pediatric nodal marginal zone lymphoma 
 Follicular lymphoma 
  In situ follicular neoplasia 
  Duodenal-type follicular lymphoma 
 BCL2-R–negative, CD23-positive follicle center lymphoma 
 Primary cutaneous follicle center lymphoma 
 Pediatric-type follicular lymphoma 
 Testicular follicular lymphoma* 
 Large B-cell lymphoma with IRF4 rearrangement* 
 Mantle cell lymphoma 
  In situ mantle cell neoplasia 
  Leukemic non-nodal mantle cell lymphoma 
 Diffuse large B-cell lymphoma, NOS 
  Germinal center B-cell subtype 
  Activated B-cell subtype 
 Large B-cell lymphoma with 11q aberration* 
 Nodular lymphocyte predominant B-cell lymphoma* 
 T cell/histiocyte-rich large B-cell lymphoma 
 Primary diffuse large B-cell lymphoma of the central nervous system 
 Primary diffuse large B-cell lymphoma of the testis* 
 Primary cutaneous diffuse large B-cell lymphoma, leg type 
 Intravascular large B-cell lymphoma 
 HHV-8 and Epstein-Barr virus–negative primary effusion-based lymphoma* 
 Epstein-Barr virus–positive mucocutaneous ulcer* 
 Epstein-Barr virus–positive diffuse large B-cell lymphoma, NOS 
 Diffuse large B-cell lymphoma associated with chronic inflammation 
  Fibrin-associated diffuse large B-cell lymphoma 
 Lymphomatoid granulomatosis 
 Epstein-Barr virus–positive polymorphic B-cell lymphoproliferative disorder, NOS* 
 ALK-positive large B-cell lymphoma 
 Plasmablastic lymphoma 
 HHV-8–associated lymphoproliferative disorders 
  Multicentric Castleman disease 
  HHV-8–positive germinotropic lymphoproliferative disorder 
  HHV-8–positive diffuse large B-cell lymphoma, NOS 
  Primary effusion lymphoma 
 Burkitt lymphoma 
 High-grade B-cell lymphoma, with MYC and BCL2 rearrangements* 
 High-grade B-cell lymphoma with MYC and BCL6 rearrangements* 
 High-grade B-cell lymphoma, NOS 
 Primary mediastinal large B-cell lymphoma 
 Mediastinal gray-zone lymphoma* 
Classic Hodgkin lymphoma 
 Nodular sclerosis classic Hodgkin lymphoma 
 Lymphocyte-rich classic Hodgkin lymphoma 
 Mixed cellularity classic Hodgkin lymphoma 
 Lymphocyte-depleted classic Hodgkin lymphoma 
Mature T-cell and NK-cell neoplasms 
 T-cell prolymphocytic leukemia 
 T-cell large granular lymphocytic leukemia 
 Chronic lymphoproliferative disorder of NK cells 
 Adult T-cell leukemia/lymphoma 
 Epstein-Barr virus–positive T-cell/NK-cell lymphoproliferative disorders of childhood* 
  Hydroa vacciniforme lymphoproliferative disorder 
   Classic 
   Systemic 
  Severe mosquito bite allergy 
  Chronic active Epstein-Barr virus disease, systemic (T-cell and NK-cell phenotype) 
  Systemic Epstein-Barr virus–positive T-cell lymphoma of childhood 
 Extranodal NK/T-cell lymphoma, nasal type 
 Aggressive NK-cell leukemia 
 Primary nodal Epstein-Barr virus–positive T-cell/NK-cell lymphoma* 
 Enteropathy-associated T-cell lymphoma 
  Type II refractory celiac disease* 
 Monomorphic epitheliotropic intestinal T-cell lymphoma 
 Intestinal T-cell lymphoma, NOS 
 Indolent clonal T-cell lymphoproliferative disorder of the gastrointestinal tract* 
 Indolent NK-cell lymphoproliferative disorder of the gastrointestinal tract* 
 Hepatosplenic T-cell lymphoma 
 Mycosis fungoides 
 Sézary syndrome 
 Primary cutaneous CD30+ T-cell lymphoproliferative disorders 
  Lymphomatoid papulosis 
  Primary cutaneous anaplastic large cell lymphoma 
 Primary cutaneous small/medium CD4+ T-cell lymphoproliferative disorder 
 Subcutaneous panniculitis-like T-cell lymphoma 
 Primary cutaneous gamma-delta T-cell lymphoma 
 Primary cutaneous acral CD8+ T-cell lymphoproliferative disorder* 
 Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma 
 Peripheral T-cell lymphoma, NOS 
 Follicular helper T-cell lymphoma* 
  Follicular helper T-cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma) 
  Follicular helper T-cell lymphoma, follicular type 
  Follicular helper T-cell lymphoma, NOS 
 Anaplastic large cell lymphoma, ALK positive 
 Anaplastic large cell lymphoma, ALK negative 
 Breast implant–associated anaplastic large cell lymphoma 
Immunodeficiency-associated lymphoproliferative disorders 
 Posttransplant lymphoproliferative disorders 
  Nondestructive posttransplant lymphoproliferative disorders 
   Plasmacytic hyperplasia posttransplant lymphoproliferative disorder 
   Infectious mononucleosis posttransplant lymphoproliferative disorder 
   Florid follicular hyperplasia posttransplant lymphoproliferative disorder 
  Polymorphic posttransplant lymphoproliferative disorder 
  Monomorphic posttransplant lymphoproliferative disorder (B-cell and T-cell/NK-cell types) 
  Classic Hodgkin lymphoma posttransplant lymphoproliferative disorder 
 Other iatrogenic immunodeficiency-associated lymphoproliferative disorders 
Histiocytic and dendritic cell neoplasms 
 Histiocytic sarcoma 
 Langerhans cell histiocytosis 
 Langerhans cell sarcoma 
 Indeterminate dendritic cell histiocytosis* 
 Interdigitating dendritic cell sarcoma* 
 ALK-positive histiocytosis* 
 Disseminated juvenile xanthogranuloma 
 Erdheim-Chester disease 
 Rosai-Dorfman-Destombes disease* 
 Follicular dendritic cell sarcoma 
 Fibroblastic reticular cell sarcoma* 
 Epstein-Barr virus–positive inflammatory follicular dendritic cell/fibroblastic reticular cell tumor* 

Italic font indicates provisional tumor entities.

*

Changes from the 2016 WHO classification.

These lesions are classified according to the lymphoma to which they correspond.

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