TUMOUR-LIKE LESIONS WITH B-CELL PREDOMINANCE 

Reactive B-cell-rich lymphoid proliferations that can mimic lymphoma 

IgG4-related disease  

Unicentric Castleman disease  

Idiopathic multicentric Castleman disease   

KSHV/HHV8-associated multicentric Castleman disease  

 

PRECURSOR B-CELL NEOPLASMS 

B-CELL LYMPHOBLASTIC LEUKAEMIAS/LYMPHOMAS 

B-lymphoblastic leukaemia/lymphoma, NOS  

B-lymphoblastic leukaemia/lymphoma with high hyperdiploidy  

B-lymphoblastic leukaemia/lymphoma with hypodiploidy  

B-lymphoblastic leukaemia/lymphoma with iAMP21  

B-lymphoblastic leukaemia/lymphoma with BCR::ABL1 fusion  

B-lymphoblastic leukaemia/lymphoma with BCR::ABL1-like features 

B-lymphoblastic leukaemia/lymphoma with KMT2A rearrangement 

B-lymphoblastic leukaemia/lymphoma with ETV6:: RUNX1 fusion 

B-lymphoblastic leukaemia/lymphoma with ETV6::RUNX1-like features 

B-lymphoblastic leukaemia/lymphoma with TCF3::PBX1 fusion  

B-lymphoblastic leukaemia/lymphoma with IGH::IL3 fusion  

B-lymphoblastic leukaemia/lymphoma with TCF3::HLF fusion   

B-lymphoblastic leukaemia/lymphoma with other defined genetic abnormalities 

 

MATURE B-CELL NEOPLASMS 

PRE-NEOPLASTIC AND NEOPLASTIC SMALL LYMPHOCYTIC PROLIFERATIONS 

Monoclonal B-cell lymphocytosis  

Chronic lymphocytic leukaemia/small lymphocytic lymphoma  

SPLENIC B-CELL LYMPHOMAS AND LEUKAEMIAS 

Hairy cell leukaemia 

Splenic marginal zone lymphoma  

Splenic diffuse red pulp small B-cell lymphoma  

Splenic B-cell lymphoma/leukaemia with prominent nucleoli  

LYMPHOPLASMACYTIC LYMPHOMA 

Lymphoplasmacytic lymphoma 

MARGINAL ZONE LYMPHOMA 

Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue 

Primary cutaneous marginal zone lymphoma  

Nodal marginal zone lymphoma  

Paediatric marginal zone lymphoma  

FOLLICULAR LYMPHOMA 

In situ follicular B-cell neoplasm  

Follicular lymphoma   

Paediatric-type follicular lymphoma  

Duodenal-type follicular lymphoma  

CUTANEOUS FOLLICLE CENTRE LYMPHOMA 

Primary cutaneous follicle centre lymphoma  

MANTLE CELL LYMPHOMA 

In situ mantle cell neoplasm   

Mantle cell lymphoma  

Leukaemic non-nodal mantle cell lymphoma  

TRANSFORMATIONS OF INDOLENT B-CELL LYMPHOMAS 

Transformations of indolent B-cell lymphomas 

LARGE B-CELL LYMPHOMAS 

Diffuse large B-cell lymphoma, NOS  

T-cell/histiocyte-rich large B-cell lymphoma  

Diffuse large B-cell lymphoma/ high grade B-cell lymphoma with MYC and BCL2 rearrangements 

ALK-positive large B-cell lymphoma  

Large B-cell lymphoma with IRF4 rearrangement   

High-grade B-cell lymphoma with 11q aberrations  

Lymphomatoid granulomatosis  

EBV-positive diffuse large B-cell lymphoma  

Diffuse large B-cell lymphoma associated with chronic inflammation 

Fibrin-associated large B-cell lymphoma  

Fluid overload-associated large B-cell lymphoma  

Plasmablastic lymphoma  

Primary large B-cell lymphoma of immune-privileged sites  

Primary cutaneous diffuse large B-cell lymphoma, leg type  

Intravascular large B-cell lymphoma  

Primary mediastinal large B-cell lymphoma  

Mediastinal grey zone lymphoma  

High-grade B-cell lymphoma, NOS  

Burkitt lymphoma 

Burkitt lymphoma   

KSHV/HHV8-ASSOCIATED B-CELL LYMPHOID PROLIFERATIONS AND LYMPHOMAS 

Primary effusion lymphoma  

KSHV/HHV8-positive diffuse large B-cell lymphoma 

KSHV/HHV8-positive germinotropic lymphoproliferative disorder 

LYMPHOID PROLIFERATIONS AND LYMPHOMAS ASSOCIATED WITH IMMUNE DEFICIENCY AND DYSREGULATION 

Hyperplasias arising in immune deficiency/dysregulation  

Polymorphic lymphoproliferative disorders arising in immune deficiency/dysregulation 

EBV-positive mucocutaneous ulcer  

Lymphomas arising in immune deficiency / dysregulation  

Inborn error of immunity-associated lymphoid proliferations and lymphomas 

HODGKIN LYMPHOMA 

Classic Hodgkin lymphoma  

Nodular lymphocyte predominant Hodgkin lymphoma  

   

PLASMA CELL NEOPLASMS AND OTHER DISEASES WITH PARAPROTEINS 

MONOCLONAL GAMMOPATHIES 

Cold agglutinin disease   

IgM monoclonal gammopathy of undetermined significance   

Non-IgM monoclonal gammopathy of undetermined significance  

Monoclonal gammopathy of renal significance   

DISEASES WITH MONOCLONAL IMMUNOGLOBULIN DEPOSITION 

Immunoglobulin-related (AL) amyloidosis  

Monoclonal immunoglobulin deposition disease  

HEAVY CHAIN DISEASES 

Mu heavy chain disease  

Gamma heavy chain disease  

Alpha heavy chain disease  

PLASMA CELL NEOPLASMS 

Plasmacytoma  

Plasma cell myeloma  

Plasma cell neoplasms with associated paraneoplastic syndrome 

-POEMS syndrome 

-TEMPI syndrome 

-AESOP syndrome 

  

TUMOUR-LIKE LESIONS WITH T-CELL PREDOMINANCE 

Kikuchi-Fujimoto disease  

Indolent T-lymphoblastic proliferation   

Autoimmune lymphoproliferative syndrome 

PRECURSOR T-CELL NEOPLASMS 

T-LYMPHOBLASTIC LEUKAEMIA/LYMPHOMA 

T-lymphoblastic leukaemia / lymphoma, NOS  

Early T-precursor lymphoblastic leukaemia / lymphoma  

MATURE T-CELL AND NK-CELL NEOPLASMS 

MATURE T-CELL AND NK-CELL LEUKAEMIAS 

T-prolymphocytic leukaemia  

T-large granular lymphocytic leukaemia  

NK-large granular lymphocytic leukaemia 

Adult T-cell leukaemia/lymphoma  

Sezary syndrome  

Aggressive NK-cell leukaemia 

PRIMARY CUTANEOUS T-CELL LYMPHOMAS 

Primary cutaneous CD4-positive small or medium T-cell lymphoproliferative disorder 

Primary cutaneous acral CD8-positive lymphoproliferative disorder  

Mycosis fungoides  

Primary cutaneous CD30-positive T-cell lymphoproliferative disorder: Lymphomatoid papulosis 

Primary cutaneous CD30-positive T-cell lymphoproliferative disorder: Primary cutaneous anaplastic large cell lymphoma 

Subcutaneous panniculitis-like T-cell lymphoma   

Primary cutaneous gamma/delta T-cell lymphoma  

Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma 

Primary cutaneous peripheral T-cell lymphoma, NOS 

INTESTINAL T-CELL AND NK-CELL LYMPHOID PROLIFERATIONS AND LYMPHOMAS 

Indolent T-cell lymphoma of the gastrointestinal tract  

Indolent NK-cell lymphoproliferative disorder of the gastrointestinal tract  

Enteropathy-associated T-cell lymphoma  

Monomorphic epitheliotropic intestinal T-cell lymphoma  

Intestinal T-cell lymphoma, NOS  

HEPATOSPLENIC T-CELL LYMPHOMA 

Hepatosplenic T-cell lymphoma 

ANAPLASTIC LARGE CELL LYMPHOMA 

ALK-positive anaplastic large cell lymphoma Anaplastic large cell lymphoma, ALK-positive 

ALK-negative anaplastic large cell lymphoma Anaplastic large cell lymphoma, ALK-negative 

Breast implant-associated anaplastic large cell lymphoma (Same) 

NODAL T-FOLLICULAR HELPER (TFH) CELL LYMPHOMA 

Nodal TFH cell lymphoma, angioimmunoblastic-type  

Nodal TFH cell lymphoma, follicular-type  

Nodal TFH cell lymphoma, NOS   

OTHER PERIPHERAL T-CELL LYMPHOMAS 

Peripheral T-cell lymphoma, not otherwise specified  

EBV-POSITIVE NK/T-CELL LYMPHOMAS 

EBV-positive nodal T- and NK-cell lymphoma  

Extranodal NK/T-cell lymphoma Extranodal  

EBV-POSITIVE T- AND NK-CELL LYMPHOID PROLIFERATIONS AND LYMPHOMAS OF CHILDHOOD 

Severe mosquito bite allergy  

Hydroa vacciniforme lymphoproliferative disorder  

Systemic chronic active EBV disease  

Systemic EBV-positive T-cell lymphoma of childhood  

Bibliografía


Alaggio R, Amador C, Anagnostopoulos I, Attygalle AD, et al. The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms. Leukemia. 2022 Jul;36(7):1720-1748. doi: 10.1038/s41375-022-01620-2.