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* |