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. 2022 Jun 22;36(7):1720–1748. doi: 10.1038/s41375-022-01620-2

Table 1.

WHO Classification of Haematolymphoid Tumours, 5th edition: B-cell lymphoid proliferations and lymphomas.

WHO Classification, 5th edition WHO Classification, revised 4th edition
Tumour-like lesions with B-cell predominance
Reactive B-cell-rich lymphoid proliferations that can mimic lymphoma Not previously included
IgG4-related disease Not previously included
Unicentric Castleman disease Not previously included
Idiopathic multicentric Castleman disease Not previously included
KSHV/HHV8-associated multicentric Castleman disease Multicentric Castleman disease
Precursor B-cell neoplasms
B-cell lymphoblastic leukaemias/lymphomas
B-lymphoblastic leukaemia/lymphoma, NOS (Same)
B-lymphoblastic leukaemia/lymphoma with high hyperdiploidy B-lymphoblastic leukaemia/lymphoma with hyperdiploidy
B-lymphoblastic leukaemia/lymphoma with hypodiploidy (Same)
B-lymphoblastic leukaemia/lymphoma with iAMP21 (Same)
B-lymphoblastic leukaemia/lymphoma with BCR::ABL1 fusion B-lymphoblastic leukaemia/lymphoma with t(9;22)(q34;q11.2); BCR-ABL1
B-lymphoblastic leukaemia/lymphoma with BCR::ABL1-like features B-lymphoblastic leukaemia/lymphoma, BCR-ABL1-like
B-lymphoblastic leukaemia/lymphoma with KMT2A rearrangement B-lymphoblastic leukaemia/lymphoma with t(v;11q23.3); KMT2A-rearranged
B-lymphoblastic leukaemia/lymphoma with ETV6::RUNX1 fusion B-lymphoblastic leukaemia/lymphoma with t(12;21)(p13.2;q22.1); ETV6-RUNX1
B-lymphoblastic leukaemia/lymphoma with ETV6::RUNX1-like features Not previously included
B-lymphoblastic leukaemia/lymphoma with TCF3::PBX1 fusion B-lymphoblastic leukaemia/lymphoma with t(1;19)(q23;p13.3); TCF3-PBX1
B-lymphoblastic leukaemia/lymphoma with IGH::IL3 fusion B-lymphoblastic leukaemia/lymphoma with t(5;14)(q31.1;q32.1); IGH/IL3
B-lymphoblastic leukaemia/lymphoma with TCF3::HLF fusion Not previously included
B-lymphoblastic leukaemia/lymphoma with other defined genetic abnormalities (Same)
Mature B-cell neoplasms
Pre-neoplastic and neoplastic small lymphocytic proliferations
Monoclonal B-cell lymphocytosis (Same)
Chronic lymphocytic leukaemia/small lymphocytic lymphoma (Same)
(Entity deleted) B-cell prolymphocytic leukaemia
Splenic B-cell lymphomas and leukaemias
Hairy cell leukaemia (Same)
Splenic marginal zone lymphoma (Same)
Splenic diffuse red pulp small B-cell lymphoma (Same)
Splenic B-cell lymphoma/leukaemia with prominent nucleoli Not previously included (encompassing hairy cell leukaemia variant and some cases of B-cell prolymphocytic leukaemia)
Lymphoplasmacytic lymphoma
Lymphoplasmacytic lymphoma (Same)
Marginal zone lymphoma
Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (Same)
Primary cutaneous marginal zone lymphoma Not previously included (originally included under “extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue”)
Nodal marginal zone lymphoma (Same)
Paediatric marginal zone lymphoma (Same)
Follicular lymphoma
In situ follicular B-cell neoplasm In situ follicular neoplasia
Follicular lymphoma (Same)
Paediatric-type follicular lymphoma (Same)
Duodenal-type follicular lymphoma (Same)
Cutaneous follicle centre lymphoma
Primary cutaneous follicle centre lymphoma (Same)
Mantle cell lymphoma
In situ mantle cell neoplasm In situ mantle cell neoplasia
Mantle cell lymphoma (Same)
Leukaemic non-nodal mantle cell lymphoma (Same)
Transformations of indolent B-cell lymphomas
Transformations of indolent B-cell lymphomas Not previously included
Large B-cell lymphomas
Diffuse large B-cell lymphoma, NOS (Same)
T-cell/histiocyte-rich large B-cell lymphoma (Same)
Diffuse large B-cell lymphoma/ high grade B-cell lymphoma with MYC and BCL2 rearrangements High-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements
ALK-positive large B-cell lymphoma (Same)
Large B-cell lymphoma with IRF4 rearrangement (Same)
High-grade B-cell lymphoma with 11q aberrations Burkitt-like lymphoma with 11q aberration
Lymphomatoid granulomatosis (Same)
EBV-positive diffuse large B-cell lymphoma EBV-positive diffuse large B-cell lymphoma, NOS
Diffuse large B-cell lymphoma associated with chronic inflammation (Same)
Fibrin-associated large B-cell lymphoma Not previously included (Previously considered a subtype of diffuse large B-cell lymphoma associated with chronic inflammation)
Fluid overload-associated large B-cell lymphoma Not previously included
Plasmablastic lymphoma (Same)
Primary large B-cell lymphoma of immune-privileged sites Not previously included, encompassing primary diffuse large B-cell lymphoma of the CNS in revised 4th edition (plus primary large B-cell lymphoma of the vitreoretina and primary large B-cell lymphoma of the testis)
Primary cutaneous diffuse large B-cell lymphoma, leg type (Same)
Intravascular large B-cell lymphoma (Same)
Primary mediastinal large B-cell lymphoma (Same)
Mediastinal grey zone lymphoma B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and classic Hodgkin lymphoma
High-grade B-cell lymphoma, NOS (Same)
Burkitt lymphoma
Burkitt lymphoma (Same)
KSHV/HHV8-associated B-cell lymphoid proliferations and lymphomas
Primary effusion lymphoma (Same)
KSHV/HHV8-positive diffuse large B-cell lymphoma HHV8-positive diffuse large B-cell lymphoma, NOS
KSHV/HHV8-positive germinotropic lymphoproliferative disorder HHV8-positive germinotropic lymphoproliferative disorder
Lymphoid proliferations and lymphomas associated with immune deficiency and dysregulation
Hyperplasias arising in immune deficiency/dysregulation Not previously included, encompassing non-destructive post-transplant lymphoproliferative disorders, among others
Polymorphic lymphoproliferative disorders arising in immune deficiency/dysregulation Not previously included, encompassing polymorphic posttransplant lymphoproliferative disorders, other iatrogenic immunodeficiency-associated lymphoproliferative disorders, among others
EBV-positive mucocutaneous ulcer (Same)
Lymphomas arising in immune deficiency / dysregulation Not previously included, encompassing monomorphic posttransplant lymphoproliferative disorders, classic Hodgkin lymphoma posttransplant lymphoproliferative disorders, lymphomas associated with HIV infection, among others
Inborn error of immunity-associated lymphoid proliferations and lymphomas Lymphoproliferative diseases associated with primary immune disorders
Hodgkin lymphoma
Classic Hodgkin lymphoma (Same)
Nodular lymphocyte predominant Hodgkin lymphoma (Same)
Plasma cell neoplasms and other diseases with paraproteins
Monoclonal gammopathies
Cold agglutinin disease Not previously included
IgM monoclonal gammopathy of undetermined significance (Same)
Non-IgM monoclonal gammopathy of undetermined significance (Same)
Monoclonal gammopathy of renal significance Not previously included
Diseases with monoclonal immunoglobulin deposition
Immunoglobulin-related (AL) amyloidosis Primary amyloidosis
Monoclonal immunoglobulin deposition disease Light chain and heavy chain deposition disease
Heavy chain diseases
Mu heavy chain disease (Same)
Gamma heavy chain disease (Same)
Alpha heavy chain disease (Same)
Plasma cell neoplasms
Plasmacytoma (Same)
Plasma cell myeloma (Same)

Plasma cell neoplasms with associated paraneoplastic syndrome

-POEMS syndrome

-TEMPI syndrome

-AESOP syndrome

(Same) Except AESOP syndrome not previously included