TY - JOUR
T1 - Immune Mechanisms in Myelodysplastic Syndrome
AU - Glenthøj, Andreas
AU - Ørskov, Andreas Due
AU - Hansen, Jakob Werner
AU - Hadrup, Sine Reker
AU - O'Connell, Casey
AU - Grønbæk, Kirsten
PY - 2016/6/15
Y1 - 2016/6/15
N2 - 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.
AB - 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.
KW - Autoimmune Diseases
KW - Autoimmunity
KW - Humans
KW - Immune System
KW - Immunity
KW - Immunomodulation
KW - Immunosuppression
KW - Immunosuppressive Agents
KW - Myelodysplastic Syndromes
KW - Journal Article
KW - Review
U2 - 10.3390/ijms17060944
DO - 10.3390/ijms17060944
M3 - Review
C2 - 27314337
SN - 1661-6596
VL - 17
JO - International Journal of Molecular Sciences (Online)
JF - International Journal of Molecular Sciences (Online)
IS - 6
M1 - 944
ER -