Immune Mechanisms in Myelodysplastic Syndrome.
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.
Epi-/Genome Laboratory, Department of Hematology, Rigshospitalet, Copenhagen University Hospital, Copenhagen 2100, Denmark. angl@rh.dk.
Andreas
A
Glenthøj
Epi-/Genome Laboratory, Department of Hematology, Rigshospitalet, Copenhagen University Hospital, Copenhagen 2100, Denmark. a.oerskov@gmail.com.
Andreas Due
AD
Ørskov
Epi-/Genome Laboratory, Department of Hematology, Rigshospitalet, Copenhagen University Hospital, Copenhagen 2100, Denmark. jakobwerner@gmail.com.
Jakob Werner
JW
Hansen
Section for Immunology and Vaccinology, National Veterinary Institute, Technical University of Denmark, Frederiksberg 1870, Denmark. sirha@vet.dtu.dk.
Sine Reker
SR
Hadrup
Jane Anne Nohl Division of Hematology, USC Norris Comprehensive Cancer Center, Los Angeles, CA 90033, USA. OConnell_C@med.usc.edu.|Stand up to Cancer Epigenetics Dream Team, Van Andel Research Institute, Grand Rapids, MI 49503, USA. OConnell_C@med.usc.edu.
Casey
C
O'Connell
Epi-/Genome Laboratory, Department of Hematology, Rigshospitalet, Copenhagen University Hospital, Copenhagen 2100, Denmark. Kirsten.Groenbaek@regionh.dk.|Stand up to Cancer Epigenetics Dream Team, Van Andel Research Institute, Grand Rapids, MI 49503, USA. Kirsten.Groenbaek@regionh.dk.
Kirsten
K
Grønbæk
D001327:Autoimmune Diseases / Q000150:complications / Q000276:immunology; D015551:Autoimmunity; D006801:Humans; D007107:Immune System / Q000166:cytology / Q000276:immunology / Q000378:metabolism; D007109:Immunity; D056747:Immunomodulation; D007165:Immunosuppression Therapy / Q000379:methods; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D009190:Myelodysplastic Syndromes / Q000209:etiology* / Q000378:metabolism / Q000628:therapy
D007166:Immunosuppressive Agents
Pancytopenia; adaptive immunity; autoimmunity; innate immunity; myelodysplastic syndrome