Table 1

2016 WHO classification of mature lymphoid, histiocytic, and dendritic neoplasms

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+ DLBCL, NOS* 
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 classical 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* 
Hodgkin lymphoma 
 Nodular lymphocyte predominant Hodgkin lymphoma 
 Classical Hodgkin lymphoma 
  Nodular sclerosis classical Hodgkin lymphoma 
  Lymphocyte-rich classical Hodgkin lymphoma 
  Mixed cellularity classical Hodgkin lymphoma 
  Lymphocyte-depleted classical Hodgkin 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) 
 Classical 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* 
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+ DLBCL, NOS* 
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 classical 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* 
Hodgkin lymphoma 
 Nodular lymphocyte predominant Hodgkin lymphoma 
 Classical Hodgkin lymphoma 
  Nodular sclerosis classical Hodgkin lymphoma 
  Lymphocyte-rich classical Hodgkin lymphoma 
  Mixed cellularity classical Hodgkin lymphoma 
  Lymphocyte-depleted classical Hodgkin 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) 
 Classical 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* 

Provisional entities are listed in italics.

*

Changes from the 2008 classification.

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