Immune Mechanisms in Myelodysplastic Syndrome

Andreas Glenthøj, Andreas Due Ørskov, Jakob Werner Hansen, Sine Reker Hadrup, Casey O'Connell, Kirsten Grønbæk

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    Abstract

    Myelodysplastic syndrome (MDS) is a spectrum of diseases, characterized by debilitating cytopenias and a propensity of developing acute myeloid leukemia. Comprehensive sequencing efforts have revealed a range of mutations characteristic, but not specific, of MDS. Epidemiologically, autoimmune diseases are common in patients with MDS, fueling hypotheses of common etiological mechanisms. Both innate and adaptive immune pathways are overly active in the hematopoietic niche of MDS. Although supportive care, growth factors, and hypomethylating agents are the mainstay of MDS treatment, some patients-especially younger low-risk patients with HLA-DR15 tissue type-demonstrate impressive response rates after immunosuppressive therapy. This is in contrast to higher-risk MDS patients, where several immune activating treatments, such as immune checkpoint inhibitors, are in the pipeline. Thus, the dual role of immune mechanisms in MDS is challenging, and rigorous translational studies are needed to establish the value of immune manipulation as a treatment of MDS.
    Original languageEnglish
    Article number944
    JournalInternational Journal of Molecular Sciences
    Volume17
    Issue number6
    ISSN1661-6596
    DOIs
    Publication statusPublished - 2016

    Keywords

    • Pancytopenia
    • adaptive immunity
    • autoimmunity
    • innate immunity
    • myelodysplastic syndrome

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