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WHO classification of haematolymphoid tumours

Changed by Frank Gaillard, 12 Mar 2023
Back to revision history
Disclosures - updated 6 Dec 2022:
  • Biogen Australia Pty Ltd, Investigator-Initiated Research Grant for CAD software in multiple sclerosis: finished Oct 2021 (past)

Updates to Article Attributes

Body was changed:

The World Health Organisation (WHO) classification of tumours of haematopoietic and lymphoid tissues is the most widely used pathologic classification system for hematolymphoid neoplasms. The current revision, known as the 5th edition, was published in 2022 and supersedes the 4th edition revised published in 2016.

IMPORTANT: We are in the process of updating this section. For now, the 2016 version forms the basisarticle below is a mixture of the article belowboth 2016 and 2022 classifications 1-3.

ClassificationMyeloid proliferations and neoplasms

Myeloid neoplasmsprecursor lesion

  • myeloproliferative neoplasms

  • mastocytosis

    • cutaneous mastocytosis

    • systemic mastocytosis

      • indolent systemic mastocytosis

      • smouldering systemic mastocytosis

      • systemic mastocytosis with an associated haematological neoplasm

      • aggressive systemic mastocytosis

      • mast cell leukaemia

    • 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 leukaemia

    • atypical chronic myeloid leukaemia, BCR-ABL1-negative

    • juvenile myelomonocytic leukaemia

    • 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 leukaemia 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 leukaemia associated with neurofibromatosis, Noonan syndrome or Noonan syndrome-like disorders

      • myeloid neoplasms associated with Down syndrome

  • acute myeloid leukaemia and related neoplasms

    • acute myeloid leukaemia with recurrent genetic abnormalities

      • acute myeloid leukaemia with t(8;21)(q22;q22.1); RUNX1-RUNX1T1

      • acute myeloid leukaemia with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFB-MYH11

      • acute promyelocytic leukaemia with PML-RARA

      • acute myeloid leukaemia with t(9;11)(p21.3;q23.3); MLLT3-KMT2A

      • acute myeloid leukaemia with t(6;9)(p23;q34.1); DEK-NUP214

      • acute myeloid leukaemia with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2); GATA2, MECOM

      • acute myeloid leukaemia (megakaryoblastic) with t(1;22)(p13.3;q13.3); RBM15-MKL1

      • acute myeloid leukaemia with BCR-ABL1 (provisional entity)

      • acute myeloid leukaemia with mutated NPM1

      • acute myeloid leukaemia with biallelic mutations of CEBPA

      • acute myeloid leukaemia with mutated RUNX1 (provisional entity)

    • acute myeloid leukaemia with myelodysplasia-related changes

    • therapy-related myeloid neoplasms

    • acute myeloid leukaemia, not otherwise specified

      • acute myeloid leukaemia with minimal differentiation

      • acute myeloid leukaemia without maturation

      • acute myeloid leukaemia with maturation

      • acute myelomonocytic leukaemia

      • acute monoblastic/monocytic leukaemia

      • pure erythroid leukaemia

      • acute megakaryoblastic leukaemia

      • acute basophilic leukaemia

      • acute panmyelosis with myelofibrosis

    • myeloid sarcoma

    • myeloid proliferations related to Down syndrome

      • transient abnormal myelopoiesis

      • myeloid leukaemia associated with Down syndrome

  • blastic plasmacytoid dendritic cell neoplasm

  • acute leukemias of ambiguous lineage

    • acute undifferentiated leukaemia

    • mixed phenotype acute leukaemia with t(9;22)(q34.1;q11.2); BCR-ABL1

    • mixed phenotype acute leukaemia with t(v;11q23.3); KMT2A rearranged

    • mixed phenotype acute leukaemia, B/myeloid, not otherwise specified

    • mixed phenotype acute leukaemia, T/myeloid, not otherwise specified

Lymphoid neoplasms
Histiocytic and dendritic cellMyeloproliferative neoplasms

See also

Mastocytosis

  • 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

  • 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 with other defined genetic alterations

  • 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, secondary

  • myeloid neoplasm post cytotoxic therapy

  • myeloid neoplasms associated with germline predisposition

  • myeloid proliferations associated with Down syndrome

Myeloid/lymphoid neoplasms

  • 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 JAK2 rearrangement

    • myeloid/lymphoid neoplasm with FLT3 rearrangement

    • myeloid/lymphoid neoplasm with ETV6::ABL1 fusion

    • myeloid/lymphoid neoplasms with other tyrosine kinase gene fusions

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

    • aute undifferentiated leukaemia

Histiocytic/dendritic cell neoplasms

Plasmacytoid dendritic cell neoplasms
Langerhans cell and other dendritic cell neoplasms
Histiocyte/macrophage neoplasms

2016 classification below this point

Lymphoid neoplasms

See also
  • -<p>The<strong> World Health Organisation (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 5<sup>th</sup> edition, was published in 2022 and supersedes the 4<sup>th </sup>edition revised published in 2016.</p><p><strong>IMPORTANT:</strong> We are in the process of updating this section. For now, the 2016 version forms the basis of the article below <sup>1-3</sup>.</p><h4>Classification</h4><h5>Myeloid neoplasms</h5><ul>
  • -<li>
  • -<p><a href="/articles/myeloproliferative-neoplasm-1">myeloproliferative neoplasms</a></p>
  • -<ul>
  • -<li><p><a href="/articles/chronic-myeloid-leukaemia">chronic myeloid leukaemia</a>, <em>BCR-ABL1</em>-positive</p></li>
  • -<li><p><a href="/articles/chronic-neutrophilic-leukemia">chronic neutrophilic leukaemia</a></p></li>
  • -<li><p><a href="/articles/polycythaemia-vera">polycythaemia vera</a></p></li>
  • -<li><p><a href="/articles/primary-myelofibrosis">primary myelofibrosis</a></p></li>
  • -<li><p><a href="/articles/essential-thrombocythemia">essential thrombocythaemia</a></p></li>
  • -<li><p>chronic eosinophilic leukaemia, not otherwise specified</p></li>
  • -<li><p>myeloproliferative neoplasm, unclassifiable</p></li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p><a href="/articles/mastocytosis">mastocytosis</a></p>
  • -<ul>
  • -<li><p>cutaneous mastocytosis</p></li>
  • -<li>
  • -<p>systemic mastocytosis</p>
  • -<ul>
  • -<li><p>indolent systemic mastocytosis</p></li>
  • -<li><p>smouldering systemic mastocytosis</p></li>
  • -<li><p>systemic mastocytosis with an associated haematological neoplasm</p></li>
  • -<li><p>aggressive systemic mastocytosis</p></li>
  • -<li><p>mast cell leukaemia</p></li>
  • -</ul>
  • -</li>
  • -<li><p>mast cell sarcoma</p></li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p>myeloid/lymphoid neoplasms with eosinophilia and gene rearrangement</p>
  • -<ul>
  • -<li><p>myeloid/lymphoid neoplasms with <em>PDGFRA </em>rearrangement</p></li>
  • -<li><p>myeloid/lymphoid neoplasms with <em>PDGFRB </em>rearrangement</p></li>
  • -<li><p>myeloid/lymphoid neoplasms with <em>FGFR1 </em>rearrangement</p></li>
  • -<li><p>myeloid/lymphoid neoplasms with <em>PCM1-JAK2 </em>(provisional entity)</p></li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p>myelodysplastic/myeloproliferative neoplasms</p>
  • -<ul>
  • -<li><p><a href="/articles/chronic-myelomonocytic-leukaemia">chronic myelomonocytic leukaemia</a></p></li>
  • -<li><p>atypical chronic myeloid leukaemia, <em>BCR-ABL1</em>-negative</p></li>
  • -<li><p>juvenile myelomonocytic leukaemia</p></li>
  • -<li><p>myelodysplastic/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis</p></li>
  • -<li><p>myelodysplastic/myeloproliferative neoplasm, unclassifiable</p></li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p><a href="/articles/myelodysplastic-syndrome">myelodysplastic syndromes</a></p>
  • -<ul>
  • -<li><p>myelodysplastic syndrome with single lineage dysplasia</p></li>
  • -<li><p>myelodysplastic syndrome with multilineage dysplasia</p></li>
  • -<li>
  • -<p>myelodysplastic syndrome with ring sideroblasts</p>
  • -<ul>
  • -<li><p>myelodysplastic syndrome with ring sideroblasts and single lineage dysplasia</p></li>
  • -<li><p>myelodysplastic syndrome with ring sideroblasts and multilineage dysplasia</p></li>
  • -</ul>
  • -</li>
  • -<li><p>myelodysplastic syndrome with excess blasts</p></li>
  • -<li><p>myelodysplastic syndrome with isolated del(5q)</p></li>
  • -<li><p>myelodysplastic syndrome, unclassifiable</p></li>
  • -<li><p>refractory cytopenia of childhood (provisional entity)</p></li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p>myeloid neoplasms with germline predisposition</p>
  • -<ul>
  • -<li>
  • -<p>myeloid neoplasms with germline predisposition without a preexisting disorder or organ dysfunction</p>
  • -<ul>
  • -<li><p>acute myeloid leukaemia with germline <em>CEBPA </em>mutation</p></li>
  • -<li><p>myeloid neoplasms with germline <em>DDX41 </em>mutation</p></li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p>myeloid neoplasms with germline predisposition and preexisting platelet disorders</p>
  • -<ul>
  • -<li><p>myeloid neoplasms with germline <em>RUNX1 </em>mutation</p></li>
  • -<li><p>myeloid neoplasms with germline <em>ANKRD26 </em>mutation</p></li>
  • -<li><p>myeloid neoplasms with germline <em>ETV6 </em>mutation</p></li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p>myeloid neoplasms with germline predisposition and other organ dysfunction</p>
  • -<ul>
  • -<li><p>myeloid neoplasms with germline <em>GATA2 </em>mutation</p></li>
  • -<li><p>myeloid neoplasms associated with bone marrow failure syndromes</p></li>
  • -<li><p>myeloid neoplasms associated with telomere biology disorders</p></li>
  • -<li><p>juvenile myelomonocytic leukaemia associated with <a href="/articles/neurofibromatosis">neurofibromatosis</a>, <a href="/articles/noonan-syndrome">Noonan syndrome</a> or Noonan syndrome-like disorders</p></li>
  • -<li><p>myeloid neoplasms associated with <a href="/articles/down-syndrome">Down syndrome</a></p></li>
  • -</ul>
  • -</li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p><a href="/articles/acute-myeloid-leukaemia">acute myeloid leukaemia</a> and related neoplasms</p>
  • -<ul>
  • -<li>
  • -<p>acute myeloid leukaemia with recurrent genetic abnormalities</p>
  • -<ul>
  • -<li><p>acute myeloid leukaemia with t(8;21)(q22;q22.1); <em>RUNX1-RUNX1T1</em></p></li>
  • -<li><p>acute myeloid leukaemia with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); <em>CBFB-MYH11</em></p></li>
  • -<li><p><a href="/articles/acute-promyelocytic-leukemia">acute promyelocytic leukaemia</a> with <em>PML-RARA</em></p></li>
  • -<li><p>acute myeloid leukaemia with t(9;11)(p21.3;q23.3); <em>MLLT3-KMT2A</em></p></li>
  • -<li><p>acute myeloid leukaemia with t(6;9)(p23;q34.1); <em>DEK-NUP214</em></p></li>
  • -<li><p>acute myeloid leukaemia with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2); <em>GATA2</em>, <em>MECOM</em></p></li>
  • -<li><p>acute myeloid leukaemia (megakaryoblastic) with t(1;22)(p13.3;q13.3); <em>RBM15-MKL1</em></p></li>
  • -<li><p>acute myeloid leukaemia with <em>BCR-ABL1</em> (provisional entity)</p></li>
  • -<li><p>acute myeloid leukaemia with mutated <em>NPM1</em></p></li>
  • -<li><p>acute myeloid leukaemia with biallelic mutations of <em>CEBPA</em></p></li>
  • -<li><p>acute myeloid leukaemia with mutated <em>RUNX1 </em>(provisional entity)</p></li>
  • -</ul>
  • -</li>
  • -<li><p>acute myeloid leukaemia with myelodysplasia-related changes</p></li>
  • -<li><p>therapy-related myeloid neoplasms</p></li>
  • -<li>
  • -<p>acute myeloid leukaemia, not otherwise specified</p>
  • -<ul>
  • -<li><p>acute myeloid leukaemia with minimal differentiation</p></li>
  • -<li><p>acute myeloid leukaemia without maturation</p></li>
  • -<li><p>acute myeloid leukaemia with maturation</p></li>
  • -<li><p>acute myelomonocytic leukaemia</p></li>
  • -<li><p>acute monoblastic/monocytic leukaemia</p></li>
  • -<li><p>pure erythroid leukaemia</p></li>
  • -<li><p>acute megakaryoblastic leukaemia</p></li>
  • -<li><p>acute basophilic leukaemia</p></li>
  • -<li><p>acute panmyelosis with myelofibrosis</p></li>
  • -</ul>
  • -</li>
  • -<li><p><a href="/articles/myeloid-sarcoma-1">myeloid sarcoma</a></p></li>
  • -<li>
  • -<p>myeloid proliferations related to Down syndrome</p>
  • -<ul>
  • -<li><p>transient abnormal myelopoiesis</p></li>
  • -<li><p>myeloid leukaemia associated with Down syndrome</p></li>
  • -</ul>
  • -</li>
  • -</ul>
  • -</li>
  • -<li><p><a href="/articles/blastic-plasmacytoid-dendritic-cell-neoplasm">blastic plasmacytoid dendritic cell neoplasm</a></p></li>
  • -<li>
  • -<p>acute leukemias of ambiguous lineage</p>
  • -<ul>
  • -<li><p>acute undifferentiated leukaemia</p></li>
  • -<li><p>mixed phenotype acute leukaemia with t(9;22)(q34.1;q11.2);<em> BCR-ABL1</em></p></li>
  • -<li><p>mixed phenotype acute leukaemia with t(v;11q23.3); <em>KMT2A </em>rearranged</p></li>
  • -<li><p>mixed phenotype acute leukaemia, B/myeloid, not otherwise specified</p></li>
  • -<li><p>mixed phenotype acute leukaemia, T/myeloid, not otherwise specified</p></li>
  • -</ul>
  • -</li>
  • -</ul><h5>Lymphoid neoplasms</h5><ul>
  • -<li>
  • -<p><a href="/articles/acute-lymphoblastic-leukaemia">precursor lymphoid neoplasms</a></p>
  • -<ul>
  • -<li>
  • -<p>B-lymphoblastic leukaemia/lymphoma</p>
  • -<ul>
  • -<li><p>B-lymphoblastic leukaemia/lymphoma, not otherwise specified</p></li>
  • -<li>
  • -<p>B-lymphoblastic leukaemia/lymphoma with recurrent genetic abnormalities</p>
  • -<ul>
  • -<li><p>B-lymphoblastic leukaemia/lymphoma with t(9;22)(q34.1;q11.2); <em>BCR-ABL1</em></p></li>
  • -<li><p>B-lymphoblastic leukaemia/lymphoma with t(v;11q23.3); <em>KMT2A</em> rearranged</p></li>
  • -<li><p>B-lymphoblastic leukaemia/lymphoma with t(12;21)(p13.2;q22.1); <em>ETV6-RUNX1</em></p></li>
  • -<li><p>B-lymphoblastic leukaemia/lymphoma with hyperdiploidy</p></li>
  • -<li><p>B-lymphoblastic leukaemia/lymphoma with hypodiploidy</p></li>
  • -<li><p>B-lymphoblastic leukaemia/lymphoma with t(5;14)(q31.1;q32.3); <em>IL3-IGH</em></p></li>
  • -<li><p>B-lymphoblastic leukaemia/lymphoma with t(1;19)(q23;p13.3); <em>TCF3-PBX1</em></p></li>
  • -<li><p>B-lymphoblastic leukaemia/lymphoma, <em>BCR-ABL1</em>–like (provisional entity)</p></li>
  • -<li><p>B-lymphoblastic leukaemia/lymphoma with iAMP21 (provisional entity)</p></li>
  • -</ul>
  • -</li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p>T-lymphoblastic leukaemia/lymphoma</p>
  • -<ul><li><p>early T-cell precursor lymphoblastic leukaemia (provisional entity)</p></li></ul>
  • -</li>
  • -<li><p>natural killer (NK) cell lymphoblastic leukaemia/lymphoma (provisional entity)</p></li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p>mature B-cell neoplasms</p>
  • -<ul>
  • -<li><p><a href="/articles/chronic-lymphocytic-leukaemia">chronic lymphocytic leukaemia</a>/<a href="/articles/small-lymphocytic-lymphoma">small lymphocytic lymphoma</a></p></li>
  • -<li><p>monoclonal B-cell lymphocytosis</p></li>
  • -<li><p>B-cell prolymphocytic leukaemia</p></li>
  • -<li><p><a href="/articles/splenic-marginal-zone-lymphoma">splenic marginal zone lymphoma</a></p></li>
  • -<li><p><a href="/articles/hairy-cell-leukaemia" title="Hairy cell leukaemia">hairy cell leukaemia</a></p></li>
  • -<li>
  • -<p><a href="/articles/splenic-lymphoma">splenic B-cell lymphoma/leukaemia, unclassifiable</a></p>
  • -<ul>
  • -<li><p>splenic diffuse red pulp small B-cell lymphoma (provisional entity)</p></li>
  • -<li><p>hairy cell leukaemia-variant (provisional entity)</p></li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p><a href="/articles/lymphoplasmacytic-lymphoma">lymphoplasmacytic lymphoma</a></p>
  • -<ul><li><p><a href="/articles/waldenstrom-macroglobulinaemia">Waldenström macroglobulinaemia</a></p></li></ul>
  • -</li>
  • -<li><p>IgM <a href="/articles/monoclonal-gammopathy-of-undetermined-significance">monoclonal gammopathy of undetermined significance</a></p></li>
  • -<li>
  • -<p>heavy-chain diseases</p>
  • -<ul>
  • -<li><p>μ heavy-chain disease</p></li>
  • -<li><p>γ heavy-chain disease</p></li>
  • -<li><p>α heavy-chain disease</p></li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p>plasma cell neoplasms</p>
  • -<ul>
  • -<li><p>non-IgM <a href="/articles/monoclonal-gammopathy-of-undetermined-significance">monoclonal gammopathy of undetermined significance</a> (IgG/A)</p></li>
  • -<li><p><a href="/articles/multiple-myeloma-1">plasma cell myeloma</a> (a.k.a. multiple myeloma)</p></li>
  • -<li>
  • -<p>plasma cell myeloma variants</p>
  • -<ul>
  • -<li><p><a href="/articles/smouldering-multiple-myeloma">smouldering (asymptomatic) plasma cell myeloma</a></p></li>
  • -<li><p><a href="/articles/non-secretory-myeloma">non-secretory myeloma</a></p></li>
  • -<li><p><a href="/articles/plasma-cell-leukaemia" title="Plasma cell leukaemia">plasma cell leukaemia</a></p></li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p><a href="/articles/plasmacytoma">plasmacytoma</a></p>
  • -<ul>
  • -<li><p><a href="/articles/solitary-bone-plasmacytoma-1">solitary plasmacytoma of bone</a></p></li>
  • -<li><p><a href="/articles/extramedullary-plasmacytoma">extraosseous plasmacytoma</a></p></li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p>monoclonal immunoglobulin deposition diseases</p>
  • -<ul>
  • -<li><p>primary <a href="/articles/amyloidosis">amyloidosis</a></p></li>
  • -<li><p><a href="/articles/light-chain-deposition-disease">light chain</a> and heavy chain deposition diseases</p></li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p>plasma cell neoplasms with associated paraneoplastic syndrome</p>
  • -<ul>
  • -<li><p><a href="/articles/poems-syndrome">POEMS syndrome</a></p></li>
  • -<li><p>TEMPI syndrome</p></li>
  • -</ul>
  • -</li>
  • -</ul>
  • -</li>
  • -<li><p><a href="/articles/malt-lymphoma">extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)</a></p></li>
  • -<li>
  • -<p>nodal <a href="/articles/marginal-zone-lymphoma">marginal zone lymphoma</a></p>
  • -<ul><li><p>paediatric nodal marginal zone lymphoma (provisional entity)</p></li></ul>
  • -</li>
  • -<li>
  • -<p><a href="/articles/follicular-lymphoma">follicular lymphoma</a></p>
  • -<ul>
  • -<li><p>testicular follicular lymphoma</p></li>
  • -<li><p>in situ follicular neoplasia</p></li>
  • -<li><p>duodenal-type follicular lymphoma</p></li>
  • -</ul>
  • -</li>
  • -<li><p>paediatric-type follicular lymphoma</p></li>
  • -<li><p>large B-cell lymphoma with <em>IRF4</em> rearrangement (provisional entity)</p></li>
  • -<li><p>primary cutaneous follicle centre lymphoma</p></li>
  • -<li>
  • -<p><a href="/articles/mantle-cell-lymphoma">mantle cell lymphoma</a></p>
  • -<ul>
  • -<li><p>leukaemic non-nodal mantle cell lymphoma</p></li>
  • -<li><p>in situ mantle cell neoplasia</p></li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p>diffuse large B-cell lymphoma, not otherwise specified</p>
  • -<ul>
  • -<li><p>germinal centre B-cell type</p></li>
  • -<li><p>activated B-cell type</p></li>
  • -</ul>
  • -</li>
  • -<li><p>T-cell/histiocyte-rich large B-cell lymphoma</p></li>
  • -<li><p><a href="/articles/lymphomas-of-the-central-nervous-system">primary diffuse large B-cell lymphoma of the central nervous system</a></p></li>
  • -<li><p>primary cutaneous diffuse large B-cell lymphoma, leg type</p></li>
  • -<li><p><a href="/articles/ebv-positive-diffuse-large-b-cell-lymphoma-nos">EBV-positive diffuse large B-cell lymphoma, not otherwise specified</a></p></li>
  • -<li><p>EBV-positive mucocutaneous ulcer (provisional entity)</p></li>
  • -<li>
  • -<p>diffuse large B-cell lymphoma associated with chronic inflammation</p>
  • -<ul><li><p>fibrin-associated diffuse large B-cell lymphoma</p></li></ul>
  • -</li>
  • -<li><p><a href="/articles/lymphomatoid-granulomatosis">lymphomatoid granulomatosis</a></p></li>
  • -<li><p><a href="/articles/primary-mediastinal-large-b-cell-lymphoma">primary mediastinal (thymic) large B-cell lymphoma</a></p></li>
  • -<li><p>intravascular large B-cell lymphoma</p></li>
  • -<li><p><a href="/articles/alk-positive-large-b-cell-lymphoma">ALK-positive large B-cell lymphoma</a></p></li>
  • -<li><p><a href="/articles/plasmablastic-lymphoma">plasmablastic lymphoma</a></p></li>
  • -<li><p><a href="/articles/primary-effusion-lymphoma">primary effusion lymphoma</a></p></li>
  • -<li>
  • -<p>HHV8-associated lymphoproliferative disorders</p>
  • -<ul>
  • -<li><p>multicentric <a href="/articles/castleman-disease">Castleman disease</a></p></li>
  • -<li><p>HHV8-positive diffuse large B-cell lymphoma, not otherwise specified (provisional entity)</p></li>
  • -<li><p>HHV8-positive germinotropic lymphoproliferative disorder</p></li>
  • -</ul>
  • -</li>
  • -<li><p><a href="/articles/burkitt-lymphoma">Burkitt lymphoma</a></p></li>
  • -<li><p>Burkitt-like lymphoma with 11q aberration (provisional entity)</p></li>
  • -<li>
  • -<p>high-grade B-cell lymphoma</p>
  • -<ul>
  • -<li><p>high-grade B-cell lymphoma with <em>MYC </em>and <em>BCL2 </em>and/or <em>BCL6 </em>rearrangements</p></li>
  • -<li><p>high-grade B-cell lymphoma, not otherwise specified</p></li>
  • -</ul>
  • -</li>
  • -<li><p>B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma</p></li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p>mature T- and NK-cell neoplasms</p>
  • -<ul>
  • -<li><p><a href="/articles/t-cell-prolymphocytic-leukaemia-1">T-cell prolymphocytic leukaemia</a></p></li>
  • -<li><p>T-cell large granular lymphocytic leukaemia</p></li>
  • -<li><p>chronic lymphoproliferative disorder of NK cells (provisional entity)</p></li>
  • -<li><p>aggressive NK-cell leukaemia</p></li>
  • -<li>
  • -<p>EBV-positive T-cell and NK-cell lymphoproliferative diseases of childhood</p>
  • -<ul>
  • -<li><p>systemic EBV-positive T-cell lymphoma of childhood</p></li>
  • -<li><p>chronic active EBV infection of T- and NK-cell type, systemic form</p></li>
  • -<li><p>hydroa vacciniforme–like lymphoproliferative disorder</p></li>
  • -<li><p>severe mosquito bite allergy</p></li>
  • -</ul>
  • -</li>
  • -<li><p>adult T-cell leukaemia/lymphoma</p></li>
  • -<li><p>extranodal NK-/T-cell lymphoma, nasal type</p></li>
  • -<li>
  • -<p>intestinal T-cell lymphoma</p>
  • -<ul>
  • -<li><p>enteropathy-associated T-cell lymphoma</p></li>
  • -<li><p>monomorphic epitheliotropic intestinal T-cell lymphoma</p></li>
  • -<li><p>intestinal T-cell lymphoma, not otherwise specified</p></li>
  • -<li><p>indolent T-cell lymphoproliferative disorder of the gastrointestinal tract (provisional entity)</p></li>
  • -</ul>
  • -</li>
  • -<li><p>hepatosplenic T-cell lymphoma</p></li>
  • -<li><p>subcutaneous panniculitis-like T-cell lymphoma</p></li>
  • -<li><p><a href="/articles/mycosis-fungoides" title="Mycosis fungoides">mycosis fungoides</a></p></li>
  • -<li><p><a href="/articles/sezary-syndrome-1">Sézary syndrome</a></p></li>
  • -<li>
  • -<p>primary cutaneous CD30+ T-cell lymphoproliferative disorders</p>
  • -<ul>
  • -<li><p>lymphomatoid papulosis</p></li>
  • -<li><p>primary cutaneous anaplastic large cell lymphoma</p></li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p>primary cutanoue peripheral T-cell lymphomas, rare subtypes</p>
  • -<ul>
  • -<li><p>primary cutaneous γδ T-cell lymphoma</p></li>
  • -<li><p>primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma (provisional entity)</p></li>
  • -<li><p>primary cutaneous acral CD8+ T-cell lymphoma (provisional entity)</p></li>
  • -<li><p>primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (provisional entity)</p></li>
  • -</ul>
  • -</li>
  • -<li><p><a href="/articles/peripheral-t-cell-lymphoma">peripheral T-cell lymphoma</a>, not otherwise specified</p></li>
  • -<li>
  • -<p>angioimmunoblastic T-cell lymphoma and other nodal lymphomas of T follicular helper cell origin</p>
  • -<ul>
  • -<li><p><a href="/articles/angioimmunoblastic-t-cell-lymphoma">angioimmunoblastic T-cell lymphoma</a></p></li>
  • -<li><p>follicular T-cell lymphoma (provisional entity)</p></li>
  • -<li><p>nodal peripheral T-cell lymphoma with T follicular helper<em> </em>phenotype (provisional entity)</p></li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p><a href="/articles/anaplastic-large-cell-lymphoma">anaplastic large-cell lymphoma</a></p>
  • -<ul>
  • -<li><p><a href="/articles/anaplastic-large-cell-lymphoma-alk-positive">anaplastic large-cell lymphoma, ALK-positive</a></p></li>
  • -<li><p><a href="/articles/anaplastic-large-cell-lymphoma-alk-negative">anaplastic large-cell lymphoma, ALK-negative</a></p></li>
  • -</ul>
  • -</li>
  • -<li><p><a href="/articles/breast-implant-associated-anaplastic-large-cell-lymphoma-2">breast implant-associated anaplastic large-cell lymphoma</a> (provisional entity)</p></li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p><a href="/articles/hodgkin-lymphoma">Hodgkin lymphoma</a></p>
  • -<ul>
  • -<li><p>nodular lymphocyte predominant Hodgkin lymphoma</p></li>
  • -<li>
  • -<p>classical Hodgkin lymphoma</p>
  • -<ul>
  • -<li><p>nodular sclerosis classical Hodgkin lymphoma</p></li>
  • -<li><p>lymphocyte-rich classical Hodgkin lymphoma</p></li>
  • -<li><p>mixed cellularity classical Hodgkin lymphoma</p></li>
  • -<li><p>lymphocyte-depleted classical Hodgkin lymphoma</p></li>
  • -</ul>
  • -</li>
  • -</ul>
  • -</li>
  • -<li>
  • -<p><a href="/articles/posttransplant-lymphoproliferative-disorder">posttransplant lymphoproliferative disorders</a> (PTLD)</p>
  • -<ul>
  • -<li><p>plasmacytic hyperplasia PTLD</p></li>
  • -<li><p>infectious mononucleosis PTLD</p></li>
  • -<li><p>florid follicular hyperplasia PTLD</p></li>
  • -<li><p>polymorphic PTLD</p></li>
  • -<li>
  • -<p>monomorphic PTLD</p>
  • -<ul>
  • -<li><p>monomorphic B-cell PTLD</p></li>
  • -<li><p>monomorphic T/NK-cell PTLD</p></li>
  • -</ul>
  • -</li>
  • -<li><p>classical Hodgkin lymphoma PTLD</p></li>
  • -</ul>
  • -</li>
  • -</ul><h5>Histiocytic and dendritic cell neoplasms</h5><ul>
  • -<li><p><a href="/articles/histiocytic-sarcoma">histiocytic sarcoma</a></p></li>
  • -<li>
  • -<p>tumours derived from Langerhans cells</p>
  • -<ul>
  • -<li><p><a href="/articles/langerhans-cell-histiocytosis">Langerhans cell histiocytosis</a></p></li>
  • -<li><p><a href="/articles/langerhans-cell-sarcoma">Langerhans cell sarcoma</a></p></li>
  • -</ul>
  • -</li>
  • -<li><p>indeterminate dendritic cell tumour</p></li>
  • -<li><p>interdigitating dendritic cell sarcoma</p></li>
  • -<li>
  • -<p>follicular dendritic cell sarcoma</p>
  • -<ul><li><p>inflammatory pseudotumour-like follicular/fibroblastic dendritic cell sarcoma</p></li></ul>
  • -</li>
  • -<li><p>fibroblastic reticular cell tumour</p></li>
  • -<li><p>disseminated juvenile xanthogranuloma</p></li>
  • -<li><p><a href="/articles/erdheim-chester-disease">Erdheim-Chester disease</a></p></li>
  • -</ul><h4>See also</h4><ul>
  • -<li><p><a href="/articles/lymphoma">lymphoma (general)</a></p></li>
  • -<li><p><a href="/articles/histiocyte-society-classification-of-histiocytoses">Histiocyte Society classification of histiocytoses</a></p></li>
  • -<li><p><a href="/articles/erdheim-chester-disease">non-Langerhans cell histiocytosis</a></p></li>
  • +<p>The<strong> World Health Organisation (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 5<sup>th</sup> edition, was published in 2022 and supersedes the 4<sup>th </sup>edition revised published in 2016.</p><p><strong>IMPORTANT:</strong> We are in the process of updating this section. For now, the article below is a mixture of both 2016 and 2022 classifications <sup>1-3</sup>.</p><h4>Myeloid proliferations and neoplasms</h4><h5>Myeloid precursor lesion</h5><ul>
  • +<li><p>clonal haematopoiesis</p></li>
  • +<li><p>clonal cytopenias of undetermined significance</p></li>
  • +</ul><h5>Myeloproliferative neoplasms</h5><ul><li>
  • +<p><a href="/articles/myeloproliferative-neoplasm-1">myeloproliferative neoplasms</a></p>
  • +<ul>
  • +<li><p><a href="/articles/chronic-myeloid-leukaemia">chronic myeloid leukaemia</a></p></li>
  • +<li><p><a href="/articles/chronic-neutrophilic-leukemia">chronic neutrophilic leukaemia</a></p></li>
  • +<li><p>chronic eosinophilic leukaemia</p></li>
  • +<li><p><a href="/articles/polycythaemia-vera">polycythaemia vera</a></p></li>
  • +<li><p><a href="/articles/essential-thrombocythemia">essential thrombocythaemia</a></p></li>
  • +<li><p><a href="/articles/primary-myelofibrosis">primary myelofibrosis</a></p></li>
  • +<li><p>juvenile myelomonocytic leukaemia</p></li>
  • +<li><p>myeloproliferative neoplasm, <a href="/articles/not-otherwise-specified-nos" title="Not otherwise specified (NOS)">NOS</a></p></li>
  • +</ul>
  • +</li></ul><h5>Mastocytosis</h5><ul><li>
  • +<p><a href="/articles/mastocytosis">mastocytosis</a></p>
  • +<ul>
  • +<li><p>cutaneous mastocytosis</p></li>
  • +<li><p>systemic mastocytosis</p></li>
  • +<li><p>mast cell sarcoma</p></li>
  • +</ul>
  • +</li></ul><h5>Myelodysplastic neoplasms</h5><ul>
  • +<li>
  • +<p>myelodysplastic neoplasms, with defining genetic abnormalities</p>
  • +<ul>
  • +<li><p>myelodysplastic neoplasm with low blasts and 5q deletion</p></li>
  • +<li><p>myelodysplastic neoplasm with low blasts and SF3B1 mutation</p></li>
  • +<li><p>myelodysplastic neoplasm with biallelic TP53 inactivation</p></li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p>myelodysplastic neoplasms, morphologically defined</p>
  • +<ul>
  • +<li><p>myelodysplastic neoplasm with low blasts</p></li>
  • +<li><p>myelodysplastic neoplasm, hypoplastic</p></li>
  • +<li><p>myelodysplastic neoplasm with increased blasts</p></li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p>myelodysplastic neoplasms of childhood</p>
  • +<ul>
  • +<li><p>childhood myelodysplastic neoplasm with low blasts</p></li>
  • +<li><p>childhood myelodysplastic neoplasm with increased blasts</p></li>
  • +</ul>
  • +</li>
  • +</ul><h5>Myelodysplastic/myeloproliferative neoplasms</h5><ul><li>
  • +<p>myelodysplastic/myeloproliferative neoplasms</p>
  • +<ul>
  • +<li><p><a href="/articles/chronic-myelomonocytic-leukaemia">chronic myelomonocytic leukaemia</a></p></li>
  • +<li><p>myelodysplastic/myeloproliferative neoplasm with neutrophilia</p></li>
  • +<li><p>myelodysplastic/myeloproliferative neoplasm with SF3B1 mutation and thrombocytosis</p></li>
  • +<li><p>myelodysplastic/myeloproliferative neoplasm, <a href="/articles/not-otherwise-specified-nos" title="Not otherwise specified (NOS)">NOS</a></p></li>
  • +</ul>
  • +</li></ul><h5>Acute myeloid leukaemia</h5><ul>
  • +<li>
  • +<p><a href="/articles/acute-myeloid-leukaemia">acute myeloid leukaemia</a> with defining genetic abnormalities</p>
  • +<ul>
  • +<li><p><a href="/articles/acute-promyelocytic-leukemia" title="Acute promyelocytic leukemia">acute promyelocytic leukaemia with <em>PML::RARA</em> fusion</a></p></li>
  • +<li><p>acute myeloid leukaemia with <em>RUNX1::RUNX1T1</em> fusion</p></li>
  • +<li><p>acute myeloid leukaemia with <em>CBFB::MYH11</em> fusion</p></li>
  • +<li><p>acute myeloid leukaemia with <em>DEK::NUP214</em> fusion</p></li>
  • +<li><p>acute myeloid leukaemia with <em>RBM15::MRTFA</em> fusion</p></li>
  • +<li><p>acute myeloid leukaemia with <em>BCR::ABL1</em> fusion</p></li>
  • +<li><p>acute myeloid leukaemia with <em>KMT2A</em> rearrangement</p></li>
  • +<li><p>acute myeloid leukaemia with <em>MECOM</em> rearrangement</p></li>
  • +<li><p>acute myeloid leukaemia with <em>NUP98</em> rearrangement</p></li>
  • +<li><p>acute myeloid leukaemia with <em>NPM1</em> mutation</p></li>
  • +<li><p>acute myeloid leukaemia with <em>CEBPA</em> mutation</p></li>
  • +<li><p>acute myeloid leukaemia, myelodysplasia-related</p></li>
  • +<li><p>acute myeloid leukaemia with other defined genetic alterations</p></li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p>acute myeloid leukaemia, defined by differentiation</p>
  • +<ul>
  • +<li><p>acute myeloid leukaemia with minimal differentiation</p></li>
  • +<li><p>acute myeloid leukaemia without maturation</p></li>
  • +<li><p>acute myeloid leukaemia with maturation</p></li>
  • +<li><p>acute basophilic leukaemia</p></li>
  • +<li><p>acute myelomonocytic leukaemia</p></li>
  • +<li><p>acute monocytic leukaemia</p></li>
  • +<li><p>acute erythroid leukaemia</p></li>
  • +<li><p>acute megakaryoblastic leukaemia</p></li>
  • +</ul>
  • +</li>
  • +<li><p><a href="/articles/myeloid-sarcoma-1">myeloid sarcoma</a></p></li>
  • +</ul><h5>Myeloid neoplasms, secondary</h5><ul>
  • +<li><p>myeloid neoplasm post cytotoxic therapy</p></li>
  • +<li><p>myeloid neoplasms associated with germline predisposition</p></li>
  • +<li><p>myeloid proliferations associated with <a href="/articles/down-syndrome">Down syndrome</a></p></li>
  • +</ul><h5>Myeloid/lymphoid neoplasms</h5><ul><li>
  • +<p>myeloid/lymphoid neoplasms with eosinophilia and gene rearrangement</p>
  • +<ul>
  • +<li><p>myeloid/lymphoid neoplasms with <em>PDGFRA </em>rearrangement</p></li>
  • +<li><p>myeloid/lymphoid neoplasms with <em>PDGFRB </em>rearrangement</p></li>
  • +<li><p>myeloid/lymphoid neoplasms with <em>FGFR1 </em>rearrangement</p></li>
  • +<li><p>myeloid/lymphoid neoplasms with <em>JAK2 </em>rearrangement</p></li>
  • +<li><p>myeloid/lymphoid neoplasm with <em>FLT3</em> rearrangement</p></li>
  • +<li><p>myeloid/lymphoid neoplasm with <em>ETV6::ABL1</em> fusion</p></li>
  • +<li><p>myeloid/lymphoid neoplasms with other tyrosine kinase gene fusions</p></li>
  • +</ul>
  • +</li></ul><h5>Acute leukaemias of mixed or ambiguous lineage</h5><ul>
  • +<li>
  • +<p>acute leukaemia of ambiguous lineage with defining genetic abnormalities</p>
  • +<ul>
  • +<li><p>mixed-phenotype acute leukaemia with BCR::ABL1 fusion</p></li>
  • +<li><p>mixed-phenotype acute leukaemia with KMT2A rearrangement</p></li>
  • +<li><p>acute leukaemia of ambiguous lineage with other defined genetic alterations</p></li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p>acute leukaemia of ambiguous lineage, immunophenotypically defined</p>
  • +<ul>
  • +<li><p>mixed-phenotype acute leukaemia, B/myeloid</p></li>
  • +<li><p>mixed-phenotype acute leukaemia, T/myeloid</p></li>
  • +<li><p>mixed-phenotype acute leukaemia, rare types</p></li>
  • +<li><p>acute leukaemia of ambiguous lineage, <a href="/articles/not-otherwise-specified-nos" title="Not otherwise specified (NOS)">NOS</a></p></li>
  • +<li><p>aute undifferentiated leukaemia</p></li>
  • +</ul>
  • +</li>
  • +</ul><h4>Histiocytic/dendritic cell neoplasms</h4><h5>Plasmacytoid dendritic cell neoplasms</h5><ul><li>
  • +<p>plasmacytoid dendritic cell neoplasms</p>
  • +<ul>
  • +<li><p>mature plasmacytoid dendritic cell proliferation associated with myeloid neoplasm</p></li>
  • +<li><p><a href="/articles/blastic-plasmacytoid-dendritic-cell-neoplasm">blastic plasmacytoid dendritic cell neoplasm</a></p></li>
  • +</ul>
  • +</li></ul><h5>Langerhans cell and other dendritic cell neoplasms</h5><ul>
  • +<li>
  • +<p>Langerhans cells neoplasms</p>
  • +<ul>
  • +<li><p><a href="/articles/langerhans-cell-histiocytosis">Langerhans cell histiocytosis</a></p></li>
  • +<li><p><a href="/articles/langerhans-cell-sarcoma">Langerhans cell sarcoma</a></p></li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p>other dendritic cell neoplasms</p>
  • +<ul>
  • +<li><p>indeterminate dendritic cell tumour</p></li>
  • +<li><p>interdigitating dendritic cell sarcoma</p></li>
  • +</ul>
  • +</li>
  • +</ul><h5>Histiocyte/macrophage neoplasms</h5><ul><li>
  • +<p>histiocytic neoplasms</p>
  • +<ul>
  • +<li><p><a href="/articles/juvenile-xanthogranuloma" title="juvenile xanthogranuloma">juvenile xanthogranuloma</a></p></li>
  • +<li><p><a href="/articles/erdheim-chester-disease">Erdheim-Chester disease</a></p></li>
  • +<li><p><a href="/articles/rosai-dorfman-disease" title="Rosai-Dorfman disease">Rosai-Dorfman Disease</a></p></li>
  • +<li><p><a href="/articles/alk-positive-histiocytosis" title="ALK-positive histiocytosis">ALK-positive histiocytosis</a></p></li>
  • +<li><p><a href="/articles/histiocytic-sarcoma">histiocytic sarcoma</a></p></li>
  • +</ul>
  • +</li></ul><p></p><h4>2016 classification below this point</h4><p></p><p></p><p></p><p></p><h5>Lymphoid neoplasms</h5><ul>
  • +<li>
  • +<p><a href="/articles/acute-lymphoblastic-leukaemia">precursor lymphoid neoplasms</a></p>
  • +<ul>
  • +<li>
  • +<p>B-lymphoblastic leukaemia/lymphoma</p>
  • +<ul>
  • +<li><p>B-lymphoblastic leukaemia/lymphoma, not otherwise specified</p></li>
  • +<li>
  • +<p>B-lymphoblastic leukaemia/lymphoma with recurrent genetic abnormalities</p>
  • +<ul>
  • +<li><p>B-lymphoblastic leukaemia/lymphoma with t(9;22)(q34.1;q11.2); <em>BCR-ABL1</em></p></li>
  • +<li><p>B-lymphoblastic leukaemia/lymphoma with t(v;11q23.3); <em>KMT2A</em> rearranged</p></li>
  • +<li><p>B-lymphoblastic leukaemia/lymphoma with t(12;21)(p13.2;q22.1); <em>ETV6-RUNX1</em></p></li>
  • +<li><p>B-lymphoblastic leukaemia/lymphoma with hyperdiploidy</p></li>
  • +<li><p>B-lymphoblastic leukaemia/lymphoma with hypodiploidy</p></li>
  • +<li><p>B-lymphoblastic leukaemia/lymphoma with t(5;14)(q31.1;q32.3); <em>IL3-IGH</em></p></li>
  • +<li><p>B-lymphoblastic leukaemia/lymphoma with t(1;19)(q23;p13.3); <em>TCF3-PBX1</em></p></li>
  • +<li><p>B-lymphoblastic leukaemia/lymphoma, <em>BCR-ABL1</em>–like (provisional entity)</p></li>
  • +<li><p>B-lymphoblastic leukaemia/lymphoma with iAMP21 (provisional entity)</p></li>
  • +</ul>
  • +</li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p>T-lymphoblastic leukaemia/lymphoma</p>
  • +<ul><li><p>early T-cell precursor lymphoblastic leukaemia (provisional entity)</p></li></ul>
  • +</li>
  • +<li><p>natural killer (NK) cell lymphoblastic leukaemia/lymphoma (provisional entity)</p></li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p>mature B-cell neoplasms</p>
  • +<ul>
  • +<li><p><a href="/articles/chronic-lymphocytic-leukaemia">chronic lymphocytic leukaemia</a>/<a href="/articles/small-lymphocytic-lymphoma">small lymphocytic lymphoma</a></p></li>
  • +<li><p>monoclonal B-cell lymphocytosis</p></li>
  • +<li><p>B-cell prolymphocytic leukaemia</p></li>
  • +<li><p><a href="/articles/splenic-marginal-zone-lymphoma">splenic marginal zone lymphoma</a></p></li>
  • +<li><p><a href="/articles/hairy-cell-leukaemia" title="Hairy cell leukaemia">hairy cell leukaemia</a></p></li>
  • +<li>
  • +<p><a href="/articles/splenic-lymphoma">splenic B-cell lymphoma/leukaemia, unclassifiable</a></p>
  • +<ul>
  • +<li><p>splenic diffuse red pulp small B-cell lymphoma (provisional entity)</p></li>
  • +<li><p>hairy cell leukaemia-variant (provisional entity)</p></li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p><a href="/articles/lymphoplasmacytic-lymphoma">lymphoplasmacytic lymphoma</a></p>
  • +<ul><li><p><a href="/articles/waldenstrom-macroglobulinaemia">Waldenström macroglobulinaemia</a></p></li></ul>
  • +</li>
  • +<li><p>IgM <a href="/articles/monoclonal-gammopathy-of-undetermined-significance">monoclonal gammopathy of undetermined significance</a></p></li>
  • +<li>
  • +<p>heavy-chain diseases</p>
  • +<ul>
  • +<li><p>μ heavy-chain disease</p></li>
  • +<li><p>γ heavy-chain disease</p></li>
  • +<li><p>α heavy-chain disease</p></li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p>plasma cell neoplasms</p>
  • +<ul>
  • +<li><p>non-IgM <a href="/articles/monoclonal-gammopathy-of-undetermined-significance">monoclonal gammopathy of undetermined significance</a> (IgG/A)</p></li>
  • +<li><p><a href="/articles/multiple-myeloma-1">plasma cell myeloma</a> (a.k.a. multiple myeloma)</p></li>
  • +<li>
  • +<p>plasma cell myeloma variants</p>
  • +<ul>
  • +<li><p><a href="/articles/smouldering-multiple-myeloma">smouldering (asymptomatic) plasma cell myeloma</a></p></li>
  • +<li><p><a href="/articles/non-secretory-myeloma">non-secretory myeloma</a></p></li>
  • +<li><p><a href="/articles/plasma-cell-leukaemia" title="Plasma cell leukaemia">plasma cell leukaemia</a></p></li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p><a href="/articles/plasmacytoma">plasmacytoma</a></p>
  • +<ul>
  • +<li><p><a href="/articles/solitary-bone-plasmacytoma-1">solitary plasmacytoma of bone</a></p></li>
  • +<li><p><a href="/articles/extramedullary-plasmacytoma">extraosseous plasmacytoma</a></p></li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p>monoclonal immunoglobulin deposition diseases</p>
  • +<ul>
  • +<li><p>primary <a href="/articles/amyloidosis">amyloidosis</a></p></li>
  • +<li><p><a href="/articles/light-chain-deposition-disease">light chain</a> and heavy chain deposition diseases</p></li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p>plasma cell neoplasms with associated paraneoplastic syndrome</p>
  • +<ul>
  • +<li><p><a href="/articles/poems-syndrome">POEMS syndrome</a></p></li>
  • +<li><p>TEMPI syndrome</p></li>
  • +</ul>
  • +</li>
  • +</ul>
  • +</li>
  • +<li><p><a href="/articles/malt-lymphoma">extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)</a></p></li>
  • +<li>
  • +<p>nodal <a href="/articles/marginal-zone-lymphoma">marginal zone lymphoma</a></p>
  • +<ul><li><p>paediatric nodal marginal zone lymphoma (provisional entity)</p></li></ul>
  • +</li>
  • +<li>
  • +<p><a href="/articles/follicular-lymphoma">follicular lymphoma</a></p>
  • +<ul>
  • +<li><p>testicular follicular lymphoma</p></li>
  • +<li><p>in situ follicular neoplasia</p></li>
  • +<li><p>duodenal-type follicular lymphoma</p></li>
  • +</ul>
  • +</li>
  • +<li><p>paediatric-type follicular lymphoma</p></li>
  • +<li><p>large B-cell lymphoma with <em>IRF4</em> rearrangement (provisional entity)</p></li>
  • +<li><p>primary cutaneous follicle centre lymphoma</p></li>
  • +<li>
  • +<p><a href="/articles/mantle-cell-lymphoma">mantle cell lymphoma</a></p>
  • +<ul>
  • +<li><p>leukaemic non-nodal mantle cell lymphoma</p></li>
  • +<li><p>in situ mantle cell neoplasia</p></li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p>diffuse large B-cell lymphoma, not otherwise specified</p>
  • +<ul>
  • +<li><p>germinal centre B-cell type</p></li>
  • +<li><p>activated B-cell type</p></li>
  • +</ul>
  • +</li>
  • +<li><p>T-cell/histiocyte-rich large B-cell lymphoma</p></li>
  • +<li><p><a href="/articles/lymphomas-of-the-central-nervous-system">primary diffuse large B-cell lymphoma of the central nervous system</a></p></li>
  • +<li><p>primary cutaneous diffuse large B-cell lymphoma, leg type</p></li>
  • +<li><p><a href="/articles/ebv-positive-diffuse-large-b-cell-lymphoma-nos">EBV-positive diffuse large B-cell lymphoma, not otherwise specified</a></p></li>
  • +<li><p>EBV-positive mucocutaneous ulcer (provisional entity)</p></li>
  • +<li>
  • +<p>diffuse large B-cell lymphoma associated with chronic inflammation</p>
  • +<ul><li><p>fibrin-associated diffuse large B-cell lymphoma</p></li></ul>
  • +</li>
  • +<li><p><a href="/articles/lymphomatoid-granulomatosis">lymphomatoid granulomatosis</a></p></li>
  • +<li><p><a href="/articles/primary-mediastinal-large-b-cell-lymphoma">primary mediastinal (thymic) large B-cell lymphoma</a></p></li>
  • +<li><p>intravascular large B-cell lymphoma</p></li>
  • +<li><p><a href="/articles/alk-positive-large-b-cell-lymphoma">ALK-positive large B-cell lymphoma</a></p></li>
  • +<li><p><a href="/articles/plasmablastic-lymphoma">plasmablastic lymphoma</a></p></li>
  • +<li><p><a href="/articles/primary-effusion-lymphoma">primary effusion lymphoma</a></p></li>
  • +<li>
  • +<p>HHV8-associated lymphoproliferative disorders</p>
  • +<ul>
  • +<li><p>multicentric <a href="/articles/castleman-disease">Castleman disease</a></p></li>
  • +<li><p>HHV8-positive diffuse large B-cell lymphoma, not otherwise specified (provisional entity)</p></li>
  • +<li><p>HHV8-positive germinotropic lymphoproliferative disorder</p></li>
  • +</ul>
  • +</li>
  • +<li><p><a href="/articles/burkitt-lymphoma">Burkitt lymphoma</a></p></li>
  • +<li><p>Burkitt-like lymphoma with 11q aberration (provisional entity)</p></li>
  • +<li>
  • +<p>high-grade B-cell lymphoma</p>
  • +<ul>
  • +<li><p>high-grade B-cell lymphoma with <em>MYC </em>and <em>BCL2 </em>and/or <em>BCL6 </em>rearrangements</p></li>
  • +<li><p>high-grade B-cell lymphoma, not otherwise specified</p></li>
  • +</ul>
  • +</li>
  • +<li><p>B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma</p></li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p>mature T- and NK-cell neoplasms</p>
  • +<ul>
  • +<li><p><a href="/articles/t-cell-prolymphocytic-leukaemia-1">T-cell prolymphocytic leukaemia</a></p></li>
  • +<li><p>T-cell large granular lymphocytic leukaemia</p></li>
  • +<li><p>chronic lymphoproliferative disorder of NK cells (provisional entity)</p></li>
  • +<li><p>aggressive NK-cell leukaemia</p></li>
  • +<li>
  • +<p>EBV-positive T-cell and NK-cell lymphoproliferative diseases of childhood</p>
  • +<ul>
  • +<li><p>systemic EBV-positive T-cell lymphoma of childhood</p></li>
  • +<li><p>chronic active EBV infection of T- and NK-cell type, systemic form</p></li>
  • +<li><p>hydroa vacciniforme–like lymphoproliferative disorder</p></li>
  • +<li><p>severe mosquito bite allergy</p></li>
  • +</ul>
  • +</li>
  • +<li><p>adult T-cell leukaemia/lymphoma</p></li>
  • +<li><p>extranodal NK-/T-cell lymphoma, nasal type</p></li>
  • +<li>
  • +<p>intestinal T-cell lymphoma</p>
  • +<ul>
  • +<li><p>enteropathy-associated T-cell lymphoma</p></li>
  • +<li><p>monomorphic epitheliotropic intestinal T-cell lymphoma</p></li>
  • +<li><p>intestinal T-cell lymphoma, not otherwise specified</p></li>
  • +<li><p>indolent T-cell lymphoproliferative disorder of the gastrointestinal tract (provisional entity)</p></li>
  • +</ul>
  • +</li>
  • +<li><p>hepatosplenic T-cell lymphoma</p></li>
  • +<li><p>subcutaneous panniculitis-like T-cell lymphoma</p></li>
  • +<li><p><a href="/articles/mycosis-fungoides" title="Mycosis fungoides">mycosis fungoides</a></p></li>
  • +<li><p><a href="/articles/sezary-syndrome-1">Sézary syndrome</a></p></li>
  • +<li>
  • +<p>primary cutaneous CD30+ T-cell lymphoproliferative disorders</p>
  • +<ul>
  • +<li><p>lymphomatoid papulosis</p></li>
  • +<li><p>primary cutaneous anaplastic large cell lymphoma</p></li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p>primary cutanoue peripheral T-cell lymphomas, rare subtypes</p>
  • +<ul>
  • +<li><p>primary cutaneous γδ T-cell lymphoma</p></li>
  • +<li><p>primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma (provisional entity)</p></li>
  • +<li><p>primary cutaneous acral CD8+ T-cell lymphoma (provisional entity)</p></li>
  • +<li><p>primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (provisional entity)</p></li>
  • +</ul>
  • +</li>
  • +<li><p><a href="/articles/peripheral-t-cell-lymphoma">peripheral T-cell lymphoma</a>, not otherwise specified</p></li>
  • +<li>
  • +<p>angioimmunoblastic T-cell lymphoma and other nodal lymphomas of T follicular helper cell origin</p>
  • +<ul>
  • +<li><p><a href="/articles/angioimmunoblastic-t-cell-lymphoma">angioimmunoblastic T-cell lymphoma</a></p></li>
  • +<li><p>follicular T-cell lymphoma (provisional entity)</p></li>
  • +<li><p>nodal peripheral T-cell lymphoma with T follicular helper<em> </em>phenotype (provisional entity)</p></li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p><a href="/articles/anaplastic-large-cell-lymphoma-alkalk">anaplastic large-cell lymphoma</a></p>
  • +<ul>
  • +<li><p>anaplastic large-cell lymphoma, ALK-positive</p></li>
  • +<li><p>anaplastic large-cell lymphoma, ALK-negative</p></li>
  • +<li><p><a href="/articles/breast-implant-associated-anaplastic-large-cell-lymphoma-2">breast implant-associated anaplastic large-cell lymphoma</a> </p></li>
  • +</ul>
  • +</li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p><a href="/articles/hodgkin-lymphoma">Hodgkin lymphoma</a></p>
  • +<ul>
  • +<li><p>nodular lymphocyte predominant Hodgkin lymphoma</p></li>
  • +<li>
  • +<p>classical Hodgkin lymphoma</p>
  • +<ul>
  • +<li><p>nodular sclerosis classical Hodgkin lymphoma</p></li>
  • +<li><p>lymphocyte-rich classical Hodgkin lymphoma</p></li>
  • +<li><p>mixed cellularity classical Hodgkin lymphoma</p></li>
  • +<li><p>lymphocyte-depleted classical Hodgkin lymphoma</p></li>
  • +</ul>
  • +</li>
  • +</ul>
  • +</li>
  • +<li>
  • +<p><a href="/articles/posttransplant-lymphoproliferative-disorder">posttransplant lymphoproliferative disorders</a> (PTLD)</p>
  • +<ul>
  • +<li><p>plasmacytic hyperplasia PTLD</p></li>
  • +<li><p>infectious mononucleosis PTLD</p></li>
  • +<li><p>florid follicular hyperplasia PTLD</p></li>
  • +<li><p>polymorphic PTLD</p></li>
  • +<li>
  • +<p>monomorphic PTLD</p>
  • +<ul>
  • +<li><p>monomorphic B-cell PTLD</p></li>
  • +<li><p>monomorphic T/NK-cell PTLD</p></li>
  • +</ul>
  • +</li>
  • +<li><p>classical Hodgkin lymphoma PTLD</p></li>
  • +</ul>
  • +</li>
  • +</ul><h5></h5><h4>See also</h4><ul>
  • +<li><p><a href="/articles/lymphoma">lymphoma (general)</a></p></li>
  • +<li><p><a href="/articles/histiocyte-society-classification-of-histiocytoses">Histiocyte Society classification of histiocytoses</a></p></li>
  • +<li><p><a href="/articles/erdheim-chester-disease">non-Langerhans cell histiocytosis</a></p></li>

References changed:

  • 1. WHO Classification of Tumours Editorial Board. Haematolymphoid tumours [Internet; beta version ahead of print]. Lyon (France): International Agency for Research on Cancer; 2022 [cited 12 Mar 2022]. (WHO classification of tumours series, 5th ed.; vol. 11). Available from: <a href="https://tumourclassification.iarc.who.int/chapters/63">https://tumourclassification.iarc.who.int/chapters/63</a>.
  • 2. Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, Bloomfield CD, Cazzola M, Vardiman JW. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. (2016) Blood. 127 (20): 2391-405. <a href="https://doi.org/10.1182/blood-2016-03-643544">doi:10.1182/blood-2016-03-643544</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/27069254">Pubmed</a> <span class="ref_v4"></span>
  • 3. Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, Advani R, Ghielmini M, Salles GA, Zelenetz AD, Jaffe ES. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. (2016) Blood. 127 (20): 2375-90. <a href="https://doi.org/10.1182/blood-2016-01-643569">doi:10.1182/blood-2016-01-643569</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/26980727">Pubmed</a> <span class="ref_v4"></span>
  • 1. Campo E, Harris NL, Pileri SA, Jaffe ES, Stein H, Thiele J. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. (2017) <a href="https://books.google.co.uk/books?vid=ISBN9789283244943">ISBN: 9789283244943</a> Available at <a href="http://publications.iarc.fr/Book-And-Report-Series/Who-Iarc-Classification-Of-Tumours/Who-Classification-Of-Tumours-Of-Haematopoietic-And-Lymphoid-Tissues-2017">IARC Publications</a>

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