Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2019 Jan;19(1):1-8.
doi: 10.1016/j.clml.2018.11.019. Epub 2018 Nov 23.

Challenges in Myelodysplastic/Myeloproliferative Neoplasms (MDS/MPN)

Brianna N Smith  1 , Michael Savona  2 , Rami S Komrokji  3
Affiliations
Review

Challenges in Myelodysplastic/Myeloproliferative Neoplasms (MDS/MPN)

Brianna N Smith et al. Clin Lymphoma Myeloma Leuk. 2019 Jan.

Abstract

Myelodysplastic/myeloproliferative neoplasms (MDS/MPN) are hybrid group of chronic myeloid neoplasms combining features of both MDS and MPN. The World Health Organization classification coined this group designation in 2008 to include chronic myelomonocytic leukemia, atypical chronic myeloid leukemia, juvenile myelomoncoytic leukemia, refractory anemia with ring sideroblasts and thrombocytosis as a provisional entity, and MDS/MPN unclassified. In this review, we highlight the challenges in diagnosing this group of the diseases, summarize the updates in classification, and discuss recent evolving understanding of the genetic landscape. We review risk-stratification models and overview the current management largely adapted from current MDS or MPN therapies. We define clinical benefit of therapy based on new proposed response criteria developed specifically for these groups of neoplasms. Finally, we introduce future opportunities including the planned international ABN MARRO (A Novel therapy combinations in untreated MDS/MPN And Relapsed/Refractory Overlap Syndromes) clinical study led by the MDS/MPN International Working Group.

Keywords: Atypical; CML; Chronic myelomonocytic leukemia; Myelodysplastic syndromes; Myeloproliferative neoplasms; Refractory anemia with ring sideroblasts and thrombocytosis.

PubMed Disclaimer

Figures

Figure 1.
Figure 1.
How do I Treat CMML? Abbreviations: AHSCT = autologous hematopoietic stem cell transplant; CMML = chronic myelomonocytic leukemia; HMA = hypomethylating agent; NGS = next generation sequencing; WHO = World Health Organization.
See this image and copyright information in PMC

References

    1. Vardiman JW, Thiele J, Arber DA, et al. The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes. Blood. 2009;114(5):937–951. - PubMed
    1. Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127(20):2391–2405. - PubMed
    1. Meggendorfer M, Roller A, Haferlach T, et al. SRSF2 mutations in 275 cases with chronic myelomonocytic leukemia (CMML). Blood. 2012. - PMC - PubMed
    1. Yoshida K, Sanada M, Shiraishi Y, et al. Frequent pathway mutations of splicing machinery in myelodysplasia. Nature. 2011;478(7367):64–69. - PubMed
    1. Savage N, George TI, Gotlib J. Myeloid neoplasms associated with eosinophilia and rearrangement of PDGFRA, PDGFRB, and FGFR1: a review. Int J Lab Hematol. 2013;35(5):491–500. - PubMed

MeSH terms