Chronic eosinophilic leukemia, ‘not otherwise specified’ (CEL-NOS)D47.-

Last updated on: 28.10.2021

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DefinitionThis section has been translated automatically.

Chronic eosinophilic leukemia (CEL-NOS) is classified as an MPN according to WHO 2016. It is a systemic disease with persistent autonomous eosinophilia > 1.5 G/l in the blood, which also affects the bone marrow and other organs.

ClassificationThis section has been translated automatically.

CEL-NOS is always a diagnosis of exclusion, present when:

  • Eosinophilia (≥ 1.5 x 109/l).
  • exclusion of BCR-ABL1 positive CML, PV, ET, PMF, CNL, CMML, aCML
  • Exclusion of rearrangements of PDGFRA, PDGFRB, FGFR1, no PCM1-JAK2, ETV6-JAK2 or BCR-JAK2 fusion gene.
  • Blasts <20% in peripheral blood and bone marrow, and inv(16)(p13.1q22), t(16;16)(p13.1;q22), t(8;21)(q22;q22.1) and lack of other diagnostic criteria for AML
  • Clonal cytogenetic or molecular alteration or blasts ≥ 2% in peripheral blood or ≥5% in bone marrow

Note(s)This section has been translated automatically.

Since clonal alterations such as TET2, ASXL1, DNMT3A mutations may also occur in a minority of elderly patients without hematologic disease, it is important to exclude other possible causes of reactive eosinophilia before making a diagnosis of CEL in the elderly based on molecular findings alone.

LiteratureThis section has been translated automatically.

  1. Arber DA et al. (2016) The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood 127:2391-2405.
  2. Barbui T et al. (2018) The 2016 WHO classification and diagnostic criteria for myeloproliferative neoplasms: document summary and in-depth discussion. Blood Cancer J 8:15.
  3. Bornhäuser M et al (2007) Concurrent JAK2(V617F) mutation and BCR-ABL translocation within committed myeloid progenitors in myelofibrosis. Leukemia 21:1824-1826.
  4. Hussein K et al. (2007) Myelofibrosis evolving during imatinib treatment of a chronic myeloproliferative disease with coexisting BCR-ABL translocation and JAK2V617F mutation. Blood109:4106-4107.
  5. Maxson JE et al (2013) Oncogenic CSF3R mutations in chronic neutrophilic leukemia and atypical CML. N Engl J Med 368:1781-1790.

Last updated on: 28.10.2021