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Encyclopedia of Pathology pp 1–10Cite as

Myelodysplastic/Myeloproliferative Neoplasms (MDS/MPN)

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Part of the book series: Encyclopedia of Pathology ((EP))

Synonyms

Atypical chronic myeloid leukemia, Philadelphia chromosome-negative (Ph1-); Chronic myelodysplastic/myeloproliferative disease; Juvenile chronic myelomonocytic leukemia; Mixed myeloproliferative/myelodysplastic syndrome; Overlap syndrome; Refractory anemia with ring sideroblasts associated with marked thrombocytosis (RARS-T)

Definition

The myelodysplastic/myeloproliferative neoplasms (MDS/MPN) are clonal myeloid neoplasms characterized at the time of their initial presentation by the simultaneous presence of myelodysplastic and myeloproliferative features, which prevent them from being classified as either myelodysplastic syndrome (MDS) or myeloproliferative neoplasm (MPN). The most common entities within the MDS/MPN group include chronic myelomonocytic leukemia (CMML), atypical chronic myeloid leukemia, BCR-ABL1negative (aCML), and juvenile myelomonocytic leukemia (JMML). A less well-defined group of MDS/MPN-like diseases include MDS/MPN unclassifiable (MDS/MPN-U) and a...

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References and Further Reading

  • Bacher, U., Haferlach, T., Schnittger, S., et al. (2011). Recent advances in diagnosis, molecular pathology and therapy of chronic myelomonocytic leukaemia. British Journal of Haematology, 153, 149–167.

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  • Itzykson, R., Kosmider, O., Renneville, A., et al. (2013). Prognostic score including gene mutations in chronic myelomonocytic leukemia. Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology, 31, 2428–2436.

    Article  CAS  Google Scholar 

  • Kohlmann, A., Grossmann, V., Klein, H. U., et al. (2010). Next-generation sequencing technology reveals a characteristic pattern of molecular mutations in 72.8% of chronic myelomonocytic leukemia by detecting frequent alterations in TET2, CBL, RAS, and RUNX1. Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology, 28, 3858–3865.

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  • Kosmider, O., Gelsi-Boyer, V., Ciudad, M., et al. (2009). TET2 gene mutation is a frequent and adverse event in chronic myelomonocytic leukemia. Haematologica, 94, 1676–1681.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Orazi, A., Bennett, J. M., Germing, U., Brunning, R. D., Bain, B. J., Cazzola, M., Foucar, K., Thiele, J., Baumann, I., Niemeyer, C. M., Hasserhian, R. P., & Malcovati, L. (2017). Myelodysplastic/myeloproliferative neoplasms. Lyon: IARC.

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  • Ricci, C., Fermo, E., Corti, S., et al. (2010). RAS mutations contribute to evolution of chronic myelomonocytic leukemia to the proliferative variant. Clinical Cancer Research, 16, 2246–2256.

    Article  CAS  PubMed  Google Scholar 

  • Wang, S. A., Hasserjian, R. P., Fox, P. S., et al. (2014). Atypical chronic myeloid leukemia is clinically distinct from unclassifiable myelodysplastic/myeloproliferative neoplasms. Blood, 123, 2645–2651.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

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Authors and Affiliations

  1. Department of Pathology and Laboratory Medicine, Weill Cornell Medical College, New York, NY, USA

    Julia T Geyer & Attilio Orazi

Authors
  1. Julia T Geyer
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  2. Attilio Orazi
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Corresponding author

Correspondence to Julia T Geyer .

Editor information

Editors and Affiliations

  1. NIJMEGEN, The Netherlands

    J.H.J.M. van Krieken

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Geyer, J.T., Orazi, A. (2018). Myelodysplastic/Myeloproliferative Neoplasms (MDS/MPN). In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_4774-1

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  • DOI: https://doi.org/10.1007/978-3-319-28845-1_4774-1

  • Received:

  • Accepted:

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

  • eBook Packages: Springer Reference MedicineReference Module Medicine

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