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WHO Classification of Hematolymphoid tumors

 

Myeloid precursor lesions

  • Clonal haematopoiesis
    • Clonal cytopenias of undetermined significance

 

Myeloproliferative neoplasms

  • Chronic myeloid leukaemia
  • Chronic neutrophilic leukaemia
  • Chronic eosinophilic leukaemia
  • Polycythaemia vera
  • Essential thrombocythaemia
  • Primary myelofibrosis
  • Juvenile myelomonocytic leukaemia
  • Myeloproliferative neoplasm, NOS

 

Mastocytosis

  • Cutaneous mastocytosis
  • Systemic mastocytosis
  • Mast cell sarcoma

 

Myelodysplastic neoplasms

  • Myelodysplastic neoplasms, with defining genetic abnormalities
    • Myelodysplastic neoplasm with low blasts and 5q deletion
    • Myelodysplastic neoplasm with low blasts and SF3B1 mutation
    • Myelodysplastic neoplasm with biallelic TP53 inactivation
  • Myelodysplastic neoplasms, morphologically defined
    • Myelodysplastic neoplasm with low blasts
    • Myelodysplastic neoplasm, hypoplastic
    • Myelodysplastic neoplasm with increased blasts
  • Myelodysplastic neoplasms of childhood
    • Childhood myelodysplastic neoplasm with low blasts
    • Childhood myelodysplastic neoplasm with increased blasts

 

Myelodysplastic/myeloproliferative neoplasms

  • Chronic myelomonocytic leukaemia
  • Myelodysplastic/myeloproliferative neoplasm with neutrophilia
  • Myelodysplastic/myeloproliferative neoplasm with SF3B1 mutation and thrombocytosis
  • Myelodysplastic/myeloproliferative neoplasm, NOS

 

Acute myeloid leukaemia

  • Acute myeloid leukaemia with defining genetic abnormalities
    • Acute promyelocytic leukaemia with PML::RARA fusion
    • Acute myeloid leukaemia with RUNX1::RUNX1T1 fusion
    • Acute myeloid leukaemia with CBFB::MYH11 fusion
    • Acute myeloid leukaemia with DEK::NUP214 fusion
    • Acute myeloid leukaemia with RBM15::MRTFA fusion
    • Acute myeloid leukaemia with BCR::ABL1 fusion
    • Acute myeloid leukaemia with KMT2A rearrangement
    • Acute myeloid leukaemia with MECOM rearrangement
    • Acute myeloid leukaemia with NUP98 rearrangement
    • Acute myeloid leukaemia with NPM1 mutation
    • Acute myeloid leukaemia with CEBPA mutation
  • Acute myeloid leukaemia, myelodysplasia-related
  • Acute myeloid leukaemia, defined by differentiation
    • Acute myeloid leukaemia with minimal differentiation
    • Acute myeloid leukaemia without maturation
    • Acute myeloid leukaemia with maturation
    • Acute basophilic leukaemia
    • Acute myelomonocytic leukaemia
    • Acute monocytic leukaemia
    • Acute erythroid leukaemia
    • Acute megakaryoblastic leukaemia
  • Myeloid sarcoma

 

Myeloid neoplasms and proliferations associated with antecedent or predisposing conditions

  • Myeloid neoplasm post cytotoxic therapy
  • Myeloid neoplasms associated with germline predisposition
  • Myeloid proliferations associated with Down syndrome

 

Myeloid/lymphoid neoplasms with eosinophilia and defining gene rearrangement

  • Myeloid/lymphoid neoplasm with PDGFRA rearrangement
  • Myeloid/lymphoid neoplasm with PDGFRB rearrangement
  • Myeloid/lymphoid neoplasm with FGFR1 rearrangement
  • Myeloid/lymphoid neoplasm with JAK2 rearrangement
  • Myeloid/lymphoid neoplasm with FLT3 rearrangement
  • Myeloid/lymphoid neoplasm with ETV6::ABL1 fusion
  • Myeloid/lymphoid neoplasms with other tyrosine kinase fusion genes

 

Acute leukaemias of mixed or ambiguous lineage

  • Acute leukaemia of ambiguous lineage with defining genetic abnormalities
    • Mixed-phenotype acute leukaemia with BCR::ABL1 fusion
    • Mixed-phenotype acute leukaemia with KMT2A rearrangement
    • Acute leukaemia of ambiguous lineage with other defined genetic alterations
  • Acute leukaemia of ambiguous lineage, immunophenotypically defined
    • Mixed-phenotype acute leukaemia, B/myeloid
    • Mixed-phenotype acute leukaemia, T/myeloid
    • Mixed-phenotype acute leukaemia, rare types
  • Acute leukaemia of ambiguous lineage, NOS
  • Acute undifferentiated leukaemia

 

Histiocytic/Dendritic cell neoplasms

  • Plasmacytoid dendritic cell neoplasms
    • Mature plasmacytoid dendritic cell proliferation associated with myeloid neoplasm
    • Blastic plasmacytoid dendritic cell neoplasm
  • Langerhans cell and other dendritic cell neoplasms
    • Langerhans cell histiocytosis
    • Langerhans cell sarcoma
    • Indeterminate dendritic cell tumour
    • Interdigitating dendritic cell sarcoma
  • Histiocyte/macrophage neoplasms
    • Juvenile xanthogranuloma
    • Erdheim-Chester disease
    • Rosai-Dorfman Disease
    • ALK-positive histiocytosis
    • Histiocytic sarcoma

 

B-cell lymphoid proliferations and lymphomas

  • Tumour like lesions with B-cell predominance
    • IgG4-related disease
    • Unicentric Castleman disease
    • Idiopathic multicentric Castleman disease
    • KSHV/HHV8-associated multicentric Castleman disease
  • Precursor B-cell neoplasms
    • B-lymphoblastic leukaemia/lymphoma
      • 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 alterations
      • B-lymphoblastic leukaemia/lymphoma, NOS
  • 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
    • Marginal zone lymphoma
      • Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue
      • Primary cutaneous marginal zone lymphoma
      • Nodal marginal zone lymphoma
      • Paediatric nodal 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
    • 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
    • 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 / multiple myeloma
    • Plasma cell neoplasms with associated paraneoplastic syndrome

 

T-cell and NK-cell lymphoid proliferations and lymphomas

  • Tumour-like lesions with T-cell predominance
    • Kikuchi-Fujimoto disease
    • Autoimmune lymphoproliferative syndrome
  • Indolent T-lymphoblastic proliferation
  • 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
    • 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 lymphoid proliferations and lymphomas
    • Primary cutaneous CD4-positive small or medium T-cell lymphoproliferative disorder
    • Primary cutaneous acral CD8-positive T-cell 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
  • Anaplastic large cell lymphoma
    • ALK-positive anaplastic large cell lymphoma
    • ALK-negative anaplastic large cell lymphoma
    • Breast implant-associated anaplastic large cell lymphoma
  • 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, NOS
  • EBV-positive NK-cell and T-cell lymphomas
    • EBV-positive nodal T- and NK-cell lymphoma
    • Extranodal NK/T-cell lymphoma
    • EBV-positive T-cell 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

 

Stroma-derived neoplasms of lymphoid tissues

  • Mesenchymal dendritic cell neoplasms
    • Follicular dendritic cell neoplasms
    • Follicular dendritic cell sarcoma
    • EBV-positive inflammatory follicular dendritic cell sarcoma
    • Fibroblastic reticular cell tumour
  • Myofibroblastic tumours
    • Myofibroblastic tumour
    • Intranodal palisaded myofibroblastoma
  • Spleen-specific vascular-stromal tumours
    • Splenic vascular-stromal tumours
    • Littoral cell angioma
    • Splenic hamartoma
    • Sclerosing angiomatoid nodular transformation (SANT) of spleen

 

Genetic tumour syndromes

  • Fanconi anaemia
  • Bloom syndrome
  • Ataxia-telangiectasia syndrome
  • RASopathies

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