WHO classification of haematolymphoid tumours

Changed by Yuranga Weerakkody, 28 Feb 2022

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

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

Classification

Myeloid neoplasms
  • myeloproliferative neoplasms
  • mastocytosis
    • cutaneous mastocytosis
    • systemic mastocytosis
      • indolent systemic mastocytosis
      • smolderingsmouldering systemic mastocytosis
      • systemic mastocytosis with an associated hematologicalhaematological neoplasm
      • aggressive systemic mastocytosis
      • mast cell leukemialeukaemia
    • 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 leukemialeukaemia
    • atypical chronic myeloid leukemialeukaemiaBCR-ABL1-negative
    • juvenile myelomonocytic leukemialeukaemia
    • 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 leukemialeukaemia 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 leukemialeukaemia associated with neurofibromatosisNoonan syndrome or Noonan syndrome-like disorders
      • myeloid neoplasms associated with Down syndrome
  • acute myeloid leukaemia and related neoplasms
    • acute myeloid leukemialeukaemia with recurrent genetic abnormalities
      • acute myeloid leukemialeukaemia with t(8;21)(q22;q22.1); RUNX1-RUNX1T1
      • acute myeloid leukemialeukaemia with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFB-MYH11
      • acute promyelocytic leukaemia with PML-RARA
      • acute myeloid leukemialeukaemia with t(9;11)(p21.3;q23.3); MLLT3-KMT2A
      • acute myeloid leukemialeukaemia with t(6;9)(p23;q34.1); DEK-NUP214
      • acute myeloid leukemialeukaemia with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2); GATA2MECOM
      • acute myeloid leukemialeukaemia (megakaryoblastic) with t(1;22)(p13.3;q13.3); RBM15-MKL1
      • acute myeloid leukemialeukaemia with BCR-ABL1 (provisional entity)
      • acute myeloid leukemialeukaemia with mutated NPM1
      • acute myeloid leukemialeukaemia with biallelic mutations of CEBPA
      • acute myeloid leukemialeukaemia with mutated RUNX1 (provisional entity)
    • acute myeloid leukemialeukaemia with myelodysplasia-related changes
    • therapy-related myeloid neoplasms
    • acute myeloid leukemialeukaemia, not otherwise specified
      • acute myeloid leukemialeukaemia with minimal differentiation
      • acute myeloid leukemialeukaemia without maturation
      • acute myeloid leukemialeukaemia with maturation
      • acute myelomonocytic leukemialeukaemia
      • acute monoblastic/monocytic leukemialeukaemia
      • pure erythroid leukemialeukaemia
      • acute megakaryoblastic leukemialeukaemia
      • acute basophilic leukemialeukaemia
      • acute panmyelosis with myelofibrosis
    • myeloid sarcoma
    • myeloid proliferations related to Down syndrome
      • transient abnormal myelopoiesis
      • myeloid leukemialeukaemia associated with Down syndrome
  • blastic plasmacytoid dendritic cell neoplasm
  • acute leukemias of ambiguous lineage
    • acute undifferentiated leukemialeukaemia
    • mixed phenotype acute leukemialeukaemia with t(9;22)(q34.1;q11.2); BCR-ABL1
    • mixed phenotype acute leukemialeukaemia with t(v;11q23.3); KMT2A rearranged
    • mixed phenotype acute leukemialeukaemia, B/myeloid, not otherwise specified
    • mixed phenotype acute leukemialeukaemia, T/myeloid, not otherwise specified
Lymphoid neoplasms
Histiocytic and dendritic cell neoplasms

See also

  • -<p>The<strong> World Health Organizatiοn (WHO) classification of tumours of hematopoietic and lymphoid tissues</strong> is the most widely used pathologic classification system for hematolymphoid neoplasms. The current revision, known as the 4<sup>th</sup> revised edition, was published in 2016 and supersedes the 4<sup>th </sup>edition published in 2008.</p><p>The 2016 version forms the basis of the article below <sup>1-</sup><sup>3</sup>.</p><h4>Classification</h4><h5>Myeloid neoplasms</h5><ul>
  • +<p>The<strong> World Health Organizatiοn (WHO) classification of tumours of haematopoietic and lymphoid tissues</strong> is the most widely used pathologic classification system for hematolymphoid neoplasms. The current revision, known as the 4<sup>th</sup> revised edition, was published in 2016 and supersedes the 4<sup>th </sup>edition published in 2008.</p><p>The 2016 version forms the basis of the article below <sup>1-</sup><sup>3</sup>.</p><h4>Classification</h4><h5>Myeloid neoplasms</h5><ul>
  • -<a href="/articles/chronic-myeloid-leukaemia">chronic myeloid leukemia</a>, <em>BCR-ABL1</em>-positive</li>
  • -<li><a href="/articles/chronic-neutrophilic-leukemia">chronic neutrophilic leukemia</a></li>
  • -<li><a href="/articles/polycythaemia-vera">polycythemia vera</a></li>
  • +<a href="/articles/chronic-myeloid-leukaemia">chronic myeloid leukaemia</a>, <em>BCR-ABL1</em>-positive</li>
  • +<li><a href="/articles/chronic-neutrophilic-leukemia">chronic neutrophilic leukaemia</a></li>
  • +<li><a href="/articles/polycythaemia-vera">polycythaemia vera</a></li>
  • -<li><a href="/articles/essential-thrombocythemia">essential thrombocythemia</a></li>
  • -<li>chronic eosinophilic leukemia, not otherwise specified</li>
  • +<li><a href="/articles/essential-thrombocythemia">essential thrombocythaemia</a></li>
  • +<li>chronic eosinophilic leukaemia, not otherwise specified</li>
  • -<li>smoldering systemic mastocytosis</li>
  • -<li>systemic mastocytosis with an associated hematological neoplasm</li>
  • +<li>smouldering systemic mastocytosis</li>
  • +<li>systemic mastocytosis with an associated haematological neoplasm</li>
  • -<li>mast cell leukemia</li>
  • +<li>mast cell leukaemia</li>
  • -<li><a href="/articles/chronic-myelomonocytic-leukaemia">chronic myelomonocytic leukemia</a></li>
  • -<li>atypical chronic myeloid leukemia, <em>BCR-ABL1</em>-negative</li>
  • -<li>juvenile myelomonocytic leukemia</li>
  • +<li><a href="/articles/chronic-myelomonocytic-leukaemia">chronic myelomonocytic leukaemia</a></li>
  • +<li>atypical chronic myeloid leukaemia, <em>BCR-ABL1</em>-negative</li>
  • +<li>juvenile myelomonocytic leukaemia</li>
  • -<li>acute myeloid leukemia with germline <em>CEBPA </em>mutation</li>
  • +<li>acute myeloid leukaemia with germline <em>CEBPA </em>mutation</li>
  • -<li>juvenile myelomonocytic leukemia associated with <a href="/articles/neurofibromatosis">neurofibromatosis</a>, <a href="/articles/noonan-syndrome">Noonan syndrome</a> or Noonan syndrome-like disorders</li>
  • +<li>juvenile myelomonocytic leukaemia associated with <a href="/articles/neurofibromatosis">neurofibromatosis</a>, <a href="/articles/noonan-syndrome">Noonan syndrome</a> or Noonan syndrome-like disorders</li>
  • -<li>acute myeloid leukemia with recurrent genetic abnormalities<ul>
  • -<li>acute myeloid leukemia with t(8;21)(q22;q22.1); <em>RUNX1-RUNX1T1</em>
  • +<li>acute myeloid leukaemia with recurrent genetic abnormalities<ul>
  • +<li>acute myeloid leukaemia with t(8;21)(q22;q22.1); <em>RUNX1-RUNX1T1</em>
  • -<li>acute myeloid leukemia with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); <em>CBFB-MYH11</em>
  • +<li>acute myeloid leukaemia with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); <em>CBFB-MYH11</em>
  • -<li>acute myeloid leukemia with t(9;11)(p21.3;q23.3); <em>MLLT3-KMT2A</em>
  • +<li>acute myeloid leukaemia with t(9;11)(p21.3;q23.3); <em>MLLT3-KMT2A</em>
  • -<li>acute myeloid leukemia with t(6;9)(p23;q34.1); <em>DEK-NUP214</em>
  • +<li>acute myeloid leukaemia with t(6;9)(p23;q34.1); <em>DEK-NUP214</em>
  • -<li>acute myeloid leukemia with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2); <em>GATA2</em>, <em>MECOM</em>
  • +<li>acute myeloid leukaemia with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2); <em>GATA2</em>, <em>MECOM</em>
  • -<li>acute myeloid leukemia (megakaryoblastic) with t(1;22)(p13.3;q13.3); <em>RBM15-MKL1</em>
  • +<li>acute myeloid leukaemia (megakaryoblastic) with t(1;22)(p13.3;q13.3); <em>RBM15-MKL1</em>
  • -<li>acute myeloid leukemia with <em>BCR-ABL1</em> (provisional entity)</li>
  • -<li>acute myeloid leukemia with mutated <em>NPM1</em>
  • +<li>acute myeloid leukaemia with <em>BCR-ABL1</em> (provisional entity)</li>
  • +<li>acute myeloid leukaemia with mutated <em>NPM1</em>
  • -<li>acute myeloid leukemia with biallelic mutations of <em>CEBPA</em>
  • +<li>acute myeloid leukaemia with biallelic mutations of <em>CEBPA</em>
  • -<li>acute myeloid leukemia with mutated <em>RUNX1 </em>(provisional entity)</li>
  • +<li>acute myeloid leukaemia with mutated <em>RUNX1 </em>(provisional entity)</li>
  • -<li>acute myeloid leukemia with myelodysplasia-related changes</li>
  • +<li>acute myeloid leukaemia with myelodysplasia-related changes</li>
  • -<li>acute myeloid leukemia, not otherwise specified<ul>
  • -<li>acute myeloid leukemia with minimal differentiation</li>
  • -<li>acute myeloid leukemia without maturation</li>
  • -<li>acute myeloid leukemia with maturation</li>
  • -<li>acute myelomonocytic leukemia</li>
  • -<li>acute monoblastic/monocytic leukemia</li>
  • -<li>pure erythroid leukemia</li>
  • -<li>acute megakaryoblastic leukemia</li>
  • -<li>acute basophilic leukemia</li>
  • +<li>acute myeloid leukaemia, not otherwise specified<ul>
  • +<li>acute myeloid leukaemia with minimal differentiation</li>
  • +<li>acute myeloid leukaemia without maturation</li>
  • +<li>acute myeloid leukaemia with maturation</li>
  • +<li>acute myelomonocytic leukaemia</li>
  • +<li>acute monoblastic/monocytic leukaemia</li>
  • +<li>pure erythroid leukaemia</li>
  • +<li>acute megakaryoblastic leukaemia</li>
  • +<li>acute basophilic leukaemia</li>
  • -<li>myeloid leukemia associated with Down syndrome</li>
  • +<li>myeloid leukaemia associated with Down syndrome</li>
  • -<li>acute undifferentiated leukemia</li>
  • -<li>mixed phenotype acute leukemia with t(9;22)(q34.1;q11.2);<em> BCR-ABL1</em>
  • +<li>acute undifferentiated leukaemia</li>
  • +<li>mixed phenotype acute leukaemia with t(9;22)(q34.1;q11.2);<em> BCR-ABL1</em>
  • -<li>mixed phenotype acute leukemia with t(v;11q23.3); <em>KMT2A </em>rearranged</li>
  • -<li>mixed phenotype acute leukemia, B/myeloid, not otherwise specified</li>
  • -<li>mixed phenotype acute leukemia, T/myeloid, not otherwise specified</li>
  • +<li>mixed phenotype acute leukaemia with t(v;11q23.3); <em>KMT2A </em>rearranged</li>
  • +<li>mixed phenotype acute leukaemia, B/myeloid, not otherwise specified</li>
  • +<li>mixed phenotype acute leukaemia, T/myeloid, not otherwise specified</li>
  • -<li>B-lymphoblastic leukemia/lymphoma<ul>
  • -<li>B-lymphoblastic leukemia/lymphoma, not otherwise specified</li>
  • -<li>B-lymphoblastic leukemia/lymphoma with recurrent genetic abnormalities<ul>
  • -<li>B-lymphoblastic leukemia/lymphoma with t(9;22)(q34.1;q11.2); <em>BCR-ABL1</em>
  • +<li>B-lymphoblastic leukaemia/lymphoma<ul>
  • +<li>B-lymphoblastic leukaemia/lymphoma, not otherwise specified</li>
  • +<li>B-lymphoblastic leukaemia/lymphoma with recurrent genetic abnormalities<ul>
  • +<li>B-lymphoblastic leukaemia/lymphoma with t(9;22)(q34.1;q11.2); <em>BCR-ABL1</em>
  • -<li>B-lymphoblastic leukemia/lymphoma with t(v;11q23.3); <em>KMT2A</em> rearranged</li>
  • -<li>B-lymphoblastic leukemia/lymphoma with t(12;21)(p13.2;q22.1); <em>ETV6-RUNX1</em>
  • +<li>B-lymphoblastic leukaemia/lymphoma with t(v;11q23.3); <em>KMT2A</em> rearranged</li>
  • +<li>B-lymphoblastic leukaemia/lymphoma with t(12;21)(p13.2;q22.1); <em>ETV6-RUNX1</em>
  • -<li>B-lymphoblastic leukemia/lymphoma with hyperdiploidy</li>
  • -<li>B-lymphoblastic leukemia/lymphoma with hypodiploidy</li>
  • -<li>B-lymphoblastic leukemia/lymphoma with t(5;14)(q31.1;q32.3); <em>IL3-IGH</em>
  • +<li>B-lymphoblastic leukaemia/lymphoma with hyperdiploidy</li>
  • +<li>B-lymphoblastic leukaemia/lymphoma with hypodiploidy</li>
  • +<li>B-lymphoblastic leukaemia/lymphoma with t(5;14)(q31.1;q32.3); <em>IL3-IGH</em>
  • -<li>B-lymphoblastic leukemia/lymphoma with t(1;19)(q23;p13.3); <em>TCF3-PBX1</em>
  • +<li>B-lymphoblastic leukaemia/lymphoma with t(1;19)(q23;p13.3); <em>TCF3-PBX1</em>
  • -<li>B-lymphoblastic leukemia/lymphoma, <em>BCR-ABL1</em>–like (provisional entity)</li>
  • -<li>B-lymphoblastic leukemia/lymphoma with iAMP21 (provisional entity)</li>
  • +<li>B-lymphoblastic leukaemia/lymphoma, <em>BCR-ABL1</em>–like (provisional entity)</li>
  • +<li>B-lymphoblastic leukaemia/lymphoma with iAMP21 (provisional entity)</li>
  • -<li>T-lymphoblastic leukemia/lymphoma<ul><li>early T-cell precursor lymphoblastic leukemia (provisional entity)</li></ul>
  • +<li>T-lymphoblastic leukaemia/lymphoma<ul><li>early T-cell precursor lymphoblastic leukaemia (provisional entity)</li></ul>
  • -<li>natural killer (NK) cell lymphoblastic leukemia/lymphoma (provisional entity)</li>
  • +<li>natural killer (NK) cell lymphoblastic leukaemia/lymphoma (provisional entity)</li>
  • -<a href="/articles/chronic-lymphocytic-leukaemia">chronic lymphocytic leukemia</a>/<a href="/articles/small-lymphocytic-lymphoma">small lymphocytic lymphoma</a>
  • +<a href="/articles/chronic-lymphocytic-leukaemia">chronic lymphocytic leukaemia</a>/<a href="/articles/small-lymphocytic-lymphoma">small lymphocytic lymphoma</a>
  • -<li>B-cell prolymphocytic leukemia</li>
  • +<li>B-cell prolymphocytic leukaemia</li>
  • -<li><a href="/articles/hairy-cell-leukemia">hairy cell leukemia</a></li>
  • +<li><a href="/articles/hairy-cell-leukemia">hairy cell leukaemia</a></li>
  • -<a href="/articles/splenic-lymphoma">splenic B-cell lymphoma/leukemia, unclassifiable</a><ul>
  • +<a href="/articles/splenic-lymphoma">splenic B-cell lymphoma/leukaemia, unclassifiable</a><ul>
  • -<li>hairy cell leukemia-variant (provisional entity)</li>
  • +<li>hairy cell leukaemia-variant (provisional entity)</li>
  • -<li>lymphoplasmacytic lymphoma<ul><li><a href="/articles/waldenstrom-macroglobulinaemia">Waldenström macroglobulinemia</a></li></ul>
  • +<li>
  • +<a title="lymphoplasmacytic lymphoma" href="/articles/lymphoplasmacytic-lymphoma">lymphoplasmacytic lymphoma</a><ul><li><a href="/articles/waldenstrom-macroglobulinaemia">Waldenström macroglobulinaemia</a></li></ul>
  • -<li><a href="/articles/smouldering-multiple-myeloma">smoldering (asymptomatic) plasma cell myeloma</a></li>
  • +<li><a href="/articles/smouldering-multiple-myeloma">smouldering (asymptomatic) plasma cell myeloma</a></li>
  • -<li><a href="/articles/plasma-cell-leukemia">plasma cell leukemia</a></li>
  • +<li><a href="/articles/plasma-cell-leukemia">plasma cell leukaemia</a></li>
  • -<li>nodal <a href="/articles/marginal-zone-lymphoma">marginal zone lymphoma</a><ul><li>pediatric nodal marginal zone lymphoma (provisional entity)</li></ul>
  • +<li>nodal <a href="/articles/marginal-zone-lymphoma">marginal zone lymphoma</a><ul><li>paediatric nodal marginal zone lymphoma (provisional entity)</li></ul>
  • -<li>pediatric-type follicular lymphoma</li>
  • +<li>paediatric-type follicular lymphoma</li>
  • -<li>primary cutaneous follicle center lymphoma</li>
  • +<li>primary cutaneous follicle centre lymphoma</li>
  • -<li>leukemic non-nodal mantle cell lymphoma</li>
  • +<li>leukaemic non-nodal mantle cell lymphoma</li>
  • -<li>germinal center B-cell type</li>
  • +<li>germinal centre B-cell type</li>
  • -<li><a href="/articles/t-cell-prolymphocytic-leukaemia-1">T-cell prolymphocytic leukemia</a></li>
  • -<li>T-cell large granular lymphocytic leukemia</li>
  • +<li><a href="/articles/t-cell-prolymphocytic-leukaemia-1">T-cell prolymphocytic leukaemia</a></li>
  • +<li>T-cell large granular lymphocytic leukaemia</li>
  • -<li>aggressive NK-cell leukemia</li>
  • +<li>aggressive NK-cell leukaemia</li>
  • -<li>adult T-cell leukemia/lymphoma</li>
  • +<li>adult T-cell leukaemia/lymphoma</li>
  • -<a title="peripheral T-cell lymphoma" href="/articles/peripheral-t-cell-lymphoma">peripheral T-cell lymphoma</a>, not otherwise specified</li>
  • +<a href="/articles/peripheral-t-cell-lymphoma">peripheral T-cell lymphoma</a>, not otherwise specified</li>
  • -<li>tumors derived from Langerhans cells<ul>
  • +<li>tumours derived from Langerhans cells<ul>
  • -<li>indeterminate dendritic cell tumor</li>
  • +<li>indeterminate dendritic cell tumour</li>
  • -<li>follicular dendritic cell sarcoma<ul><li>inflammatory pseudotumor-like follicular/fibroblastic dendritic cell sarcoma</li></ul>
  • +<li>follicular dendritic cell sarcoma<ul><li>inflammatory pseudotumour-like follicular/fibroblastic dendritic cell sarcoma</li></ul>
  • -<li>fibroblastic reticular cell tumor</li>
  • +<li>fibroblastic reticular cell tumour</li>

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