Table of contents :

  • chronic myelomonocytic leukemia (CMML) with eosinophilia
  • atypical chronic myeloid leukemia (aCML)
  • juvenile myelomonocytic leukemia (JMML)
  • myelodysplastic/myeloproliferative disease, unclassifiable

  • The MDS/MPD category includes myeloid disorders that have both dysplastic and proliferative features at the time of initial presentation and that are difficult to assign to either the myelodysplastic or myeloproliferative group of diseases. The WHO classification provides a less restrictive view of these diseases than do previous classification schemes that arbitrarily assigned them to either the MDS or MPD categoryref1, ref2. For individual patients, the clinician may view the patient in the context of whether proliferative or dysplastic manifestations predominate and treat accordingly. For cooperative groups and clinical investigators, the WHO category of MDS/MPD may allow for more focused clinical and scientific investigation of these entities that overlap 2 major disease categories. The criteria for the recognition of these disorders are detailed in the WHO monograph, but some aspects of CMML and aCML are particularly problematic and warrant some comment here. The molecular pathogenesis of MPD/MDS is poorly understood, but a subset of patients presents with translocations that disrupt and constitutively activate protein tyrosine kinases, most commonly the platelet-derived growth factor  receptor or the fibroblast growth factor receptor 1 (FGFR1)ref1, ref2, ref3, ref4, ref5, ref6, ref7, ref8.

    If future studies provide more definitive evidence that CMML, atypical CML, or JMML are more accurately classified as purely myelodysplastic or myeloproliferative processes, appropriate changes in their classification will be warranted.

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