WHO classification of haematolymphoid tumors

The World Health Organization (WHO) classification of tumours of hematopoietic and lymphoid tissues is the most widely used pathologic classification system for hematolymphoid neoplasms. The current revision, known as the 4^th revised edition, was published in 2016 and supersedes the 4^th edition published in 2008.

The current 2016 version forms the basis of the article below ^1-3.

Classification

Myeloid neoplasms

• myeloproliferative neoplasms
  • chronic myeloid leukemia, BCR-ABL1-positive
  • chronic neutrophilic leukemia
  • polycythemia vera
  • primary myelofibrosis
  • essential thrombocythemia
  • chronic eosinophilic leukemia, not otherwise specified
  • myeloproliferative neoplasm, unclassifiable
• mastocytosis
  • cutaneous mastocytosis
  • systemic mastocytosis
    • indolent systemic mastocytosis
    • smoldering systemic mastocytosis
    • systemic mastocytosis with an associated hematological neoplasm
    • aggressive systemic mastocytosis
    • mast cell leukemia
  • mast cell sarcoma
• myeloid/lymphoid neoplasms with eosinophilia and gene rearrangement
  • myeloid/lymphoid neoplasms with PDGFRA rearrangement
  • myeloid/lymphoid neoplasms with PDGFRB rearrangement
  • myeloid/lymphoid neoplasms with FGFR1 rearrangement
  • myeloid/lymphoid neoplasms with PCM1-JAK2 (provisional entity)
• myelodysplastic/myeloproliferative neoplasms
  • chronic myelomonocytic leukemia
  • atypical chronic myeloid leukemia, BCR-ABL1-negative
  • juvenile myelomonocytic leukemia
  • myelodysplastic/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis
  • myelodysplastic/myeloproliferative neoplasm, unclassifiable
• myelodysplastic syndromes
  • myelodysplastic syndrome with single lineage dysplasia
  • myelodysplastic syndrome with multilineage dysplasia
  • myelodysplastic syndrome with ring sideroblasts
    • myelodysplastic syndrome with ring sideroblasts and single lineage dysplasia
    • myelodysplastic syndrome with ring sideroblasts and multilineage dysplasia
  • myelodysplastic syndrome with excess blasts
  • myelodysplastic syndrome with isolated del(5q)
  • myelodysplastic syndrome, unclassifiable
  • refractory cytopenia of childhood (provisional entity)
• myeloid neoplasms with germline predisposition
  • myeloid neoplasms with germline predisposition without a preexisting disorder or organ dysfunction
    • acute myeloid leukemia with germline CEBPA mutation
    • myeloid neoplasms with germline DDX41 mutation
  • myeloid neoplasms with germline predisposition and preexisting platelet disorders
    • myeloid neoplasms with germline RUNX1 mutation
    • myeloid neoplasms with germline ANKRD26 mutation
    • myeloid neoplasms with germline ETV6 mutation
  • myeloid neoplasms with germline predisposition and other organ dysfunction
    • myeloid neoplasms with germline GATA2 mutation
    • myeloid neoplasms associated with bone marrow failure syndromes
    • myeloid neoplasms associated with telomere biology disorders
    • juvenile myelomonocytic leukemia associated with neurofibromatosis, Noonan syndrome or Noonan syndrome-like disorders
    • myeloid neoplasms associated with Down syndrome
• acute myeloid leukaemia and related neoplasms
  • acute myeloid leukemia with recurrent genetic abnormalities
    • acute myeloid leukemia with t(8;21)(q22;q22.1); RUNX1-RUNX1T1
    • acute myeloid leukemia with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFB-MYH11
    • acute promyelocytic leukaemia with PML-RARA
    • acute myeloid leukemia with t(9;11)(p21.3;q23.3); MLLT3-KMT2A
    • acute myeloid leukemia with t(6;9)(p23;q34.1); DEK-NUP214
    • acute myeloid leukemia with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2); GATA2, MECOM
    • acute myeloid leukemia (megakaryoblastic) with t(1;22)(p13.3;q13.3); RBM15-MKL1
    • acute myeloid leukemia with BCR-ABL1 (provisional entity)
    • acute myeloid leukemia with mutated NPM1
    • acute myeloid leukemia with biallelic mutations of CEBPA
    • acute myeloid leukemia with mutated RUNX1 (provisional entity)
  • acute myeloid leukemia with myelodysplasia-related changes
  • therapy-related myeloid neoplasms
  • acute myeloid leukemia, not otherwise specified
    • acute myeloid leukemia with minimal differentiation
    • acute myeloid leukemia without maturation
    • acute myeloid leukemia with maturation
    • acute myelomonocytic leukemia
    • acute monoblastic/monocytic leukemia
    • pure erythroid leukemia
    • acute megakaryoblastic leukemia
    • acute basophilic leukemia
    • acute panmyelosis with myelofibrosis
  • myeloid sarcoma
  • myeloid proliferations related to Down syndrome
    • transient abnormal myelopoiesis
    • myeloid leukemia associated with Down syndrome
• blastic plasmacytoid dendritic cell neoplasm
• acute leukemias of ambiguous lineage
  • acute undifferentiated leukemia
  • mixed phenotype acute leukemia with t(9;22)(q34.1;q11.2); BCR-ABL1
  • mixed phenotype acute leukemia with t(v;11q23.3); KMT2A rearranged
  • mixed phenotype acute leukemia, B/myeloid, not otherwise specified
  • mixed phenotype acute leukemia, T/myeloid, not otherwise specified

Lymphoid neoplasms

• precursor lymphoid neoplasms
  • B-lymphoblastic leukemia/lymphoma
    • B-lymphoblastic leukemia/lymphoma, not otherwise specified
    • B-lymphoblastic leukemia/lymphoma with recurrent genetic abnormalities
      • B-lymphoblastic leukemia/lymphoma with t(9;22)(q34.1;q11.2); BCR-ABL1
      • B-lymphoblastic leukemia/lymphoma with t(v;11q23.3); KMT2A rearranged
      • B-lymphoblastic leukemia/lymphoma with t(12;21)(p13.2;q22.1); ETV6-RUNX1
      • B-lymphoblastic leukemia/lymphoma with hyperdiploidy
      • B-lymphoblastic leukemia/lymphoma with hypodiploidy
      • B-lymphoblastic leukemia/lymphoma with t(5;14)(q31.1;q32.3); IL3-IGH
      • B-lymphoblastic leukemia/lymphoma with t(1;19)(q23;p13.3); TCF3-PBX1
      • B-lymphoblastic leukemia/lymphoma, BCR-ABL1–like (provisional entity)
      • B-lymphoblastic leukemia/lymphoma with iAMP21 (provisional entity)
  • T-lymphoblastic leukemia/lymphoma
    • early T-cell precursor lymphoblastic leukemia (provisional entity)
  • natural killer (NK) cell lymphoblastic leukemia/lymphoma (provisional entity)
• 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 (provisional entity)
    • hairy cell leukemia-variant (provisional entity)
  • lymphoplasmacytic lymphoma
    • Waldenström macroglobulinemia
  • IgM monoclonal gammopathy of undetermined significance
  • heavy-chain diseases
    • μ heavy-chain disease
    • γ heavy-chain disease
    • α heavy-chain disease
  • plasma cell neoplasms
    • non-IgM monoclonal gammopathy of undetermined significance (IgG/A)
    • plasma cell myeloma (a.k.a. multiple myeloma)
    • plasma cell myeloma variants
      • smoldering (asymptomatic) plasma cell myeloma
      • non-secretory myeloma
      • plasma cell leukemia
    • plasmacytoma
      • solitary plasmacytoma of bone
      • extraosseous plasmacytoma
    • monoclonal immunoglobulin deposition diseases
      • primary amyloidosis
      • light chain and heavy chain deposition diseases
    • plasma cell neoplasms with associated paraneoplastic syndrome
      • POEMS syndrome
      • TEMPI syndrome
  • extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)
  • nodal marginal zone lymphoma
    • pediatric nodal marginal zone lymphoma (provisional entity)
  • follicular lymphoma
    • testicular follicular lymphoma
    • in situ follicular neoplasia
    • duodenal-type follicular lymphoma
  • pediatric-type follicular lymphoma
  • large B-cell lymphoma with IRF4 rearrangement (provisional entity)
  • primary cutaneous follicle center lymphoma
  • mantle cell lymphoma
    • leukemic non-nodal mantle cell lymphoma
    • in situ mantle cell neoplasia
  • diffuse large B-cell lymphoma, not otherwise specified
    • germinal center B-cell type
    • activated B-cell type
  • T-cell/histiocyte-rich large B-cell lymphoma
  • primary diffuse large B-cell lymphoma of the central nervous system
  • primary cutaneous diffuse large B-cell lymphoma, leg type
  • EBV-positive diffuse large B-cell lymphoma, not otherwise specified
  • EBV-positive mucocutaneous ulcer (provisional entity)
  • diffuse large B-cell lymphoma associated with chronic inflammation
    • fibrin-associated diffuse large B-cell lymphoma
  • lymphomatoid granulomatosis
  • primary mediastinal (thymic) large B-cell lymphoma
  • intravascular large B-cell lymphoma
  • ALK-positive large B-cell lymphoma
  • plasmablastic lymphoma
  • primary effusion lymphoma
  • HHV8-associated lymphoproliferative disorders
    • multicentric Castleman disease
    • HHV8-positive diffuse large B-cell lymphoma, not otherwise specified (provisional entity)
    • HHV8-positive germinotropic lymphoproliferative disorder
  • Burkitt lymphoma
  • Burkitt-like lymphoma with 11q aberration (provisional entity)
  • high-grade B-cell lymphoma
    • high-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements
    • high-grade B-cell lymphoma, not otherwise specified
  • B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma
• mature T- and NK-cell neoplasms
  • T-cell prolymphocytic leukemia
  • T-cell large granular lymphocytic leukemia
  • chronic lymphoproliferative disorder of NK cells (provisional entity)
  • aggressive NK-cell leukemia
  • EBV-positive T-cell and NK-cell lymphoproliferative diseases of childhood
    • systemic EBV-positive T-cell lymphoma of childhood
    • chronic active EBV infection of T- and NK-cell type, systemic form
    • hydroa vacciniforme–like lymphoproliferative disorder
    • severe mosquito bite allergy
  • adult T-cell leukemia/lymphoma
  • extranodal NK-/T-cell lymphoma, nasal type
  • intestinal T-cell lymphoma
    • enteropathy-associated T-cell lymphoma
    • monomorphic epitheliotropic intestinal T-cell lymphoma
    • intestinal T-cell lymphoma, not otherwise specified
    • indolent T-cell lymphoproliferative disorder of the gastrointestinal tract (provisional entity)
  • 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 cutanoue peripheral T-cell lymphomas, rare subtypes
    • primary cutaneous γδ T-cell lymphoma
    • primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma (provisional entity)
    • primary cutaneous acral CD8+ T-cell lymphoma (provisional entity)
    • primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (provisional entity)
  • peripheral T-cell lymphoma, not otherwise specified
  • angioimmunoblastic T-cell lymphoma and other nodal lymphomas of T follicular helper cell origin
    • angioimmunoblastic T-cell lymphoma
    • follicular T-cell lymphoma (provisional entity)
    • nodal peripheral T-cell lymphoma with T follicular helperphenotype (provisional entity)
  • anaplastic large-cell lymphoma
    • anaplastic large-cell lymphoma, ALK-positive
    • anaplastic large-cell lymphoma, ALK-negative
  • breast implant-associated anaplastic large-cell lymphoma (provisional entity)
• 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
    • monomorphic B-cell PTLD
    • monomorphic T/NK-cell PTLD
  • classical Hodgkin lymphoma PTLD

Histiocytic and dendritic cell neoplasms

• histiocytic sarcoma
• tumors derived from Langerhans cells
  • Langerhans cell histiocytosis
  • Langerhans cell sarcoma
• indeterminate dendritic cell tumor
• interdigitating dendritic cell sarcoma
• follicular dendritic cell sarcoma
  • inflammatory pseudotumor-like follicular/fibroblastic dendritic cell sarcoma
• fibroblastic reticular cell tumor
• disseminated juvenile xanthogranuloma
• Erdheim-Chester disease

See also

• lymphoma (general)
• Histiocyte Society classification of histiocytoses
• non-Langerhans cell histiocytosis