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

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
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
Follicular lymphoma
  • In situ follicular neoplasia*
  • Duodenal-type follicular lymphoma*
Pediatric-type follicular lymphoma*
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*
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
Burkitt lymphoma
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
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*
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
Peripheral T cell lymphoma, NOS
Angioimmunoblastic T cell lymphoma
Anaplastic large cell lymphoma, ALK+
Anaplastic large cell lymphoma, ALK*
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
Post-transplant 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 not listed.
* Changes from the 2008 classification.
Modified with permission of the American Society of Hematology, from Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 2016; 127:2375. Copyright © 2016; permission conveyed through Copyright Clearance Center, Inc.
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