The immune landscape of myelodysplastic syndromes
Even though the pathogenesis of myelodysplastic syndromes (MDS) is dominated by specific molecular defects involving hematopoietic precursors, also immune mechanisms seem to play a fundamental functional role. In this review we will first describe the clinical and laboratory autoimmune manifestations often detectable in MDS patients. We will then focus on studies addressing the possible influence of different immune cell subpopulations on the disease onset and evolution. We will finally consider therapeutic approaches based on immunomodulation, ranging from immunosuppressants to vaccination and transplantation strategies.
This study was aimed to analyze the prevalence, clinical characteristics, and outcomes of MDS with AD in a series of 142 patients diagnosed with MDS and CMML. AD was ascertained by both the presence of clinical symptoms or compatible serological tests. In total, 48% patients were diagnosed as having AD, being hypothyroidism the most commonly reported clinical AD (8%) and antinuclear antibodies the most frequent serological parameter identified (23.2%). The presence of AD was associated with female gender, lower hemoglobin levels, and higher IPSS-R. Overall survival for patients with AD was inferior to those with no AD (69 ...
In this study, we aimed to characterize patient- and disease-based differences in MDS/CMML patients with and without SIAMs and explore the impact of SIAMs on progression and survival.
Authors: Senjo H, Higuchi T, Morimoto M, Koyamada R, Yanaoka C, Okada S Abstract An 81-year-old Japanese man presented with constitutional symptoms and anemia and was diagnosed with giant cell arteritis (GCA) and myelodysplastic syndrome (MDS) simultaneously. His symptoms and anemia improved promptly with steroids; however, the MDS rapidly progressed to overt leukemia. While MDS patients are at an increased risk of autoimmune diseases, an association with GCA has rarely been reported. This case illustrates the importance of considering GCA as a cause of anemia in elderly patients if MDS is already diagnosed, even i...
In conclusion, our results demonstrate that CD8+ T cells and IFN-γ are associated with autoimmune myelofibrosis, a finding that may allow targeting of CD8+ T cells and IFN-γ as a therapeutic targets. PMID: 29307587 [PubMed - as supplied by publisher]
Myelodysplastic syndrome (MDS) is a heterogeneous hematological malignancy, characterized by cytopenia and accompanied by a risk of transformation into acute myeloid leukemia (AML). Epidemiological studies for decades have shown association between autoimmune diseases (AIDs) and MDS. Specifically, patients with antecedent AIDs tends to have an increased risk of developing MDS, and these patients display different clinical characteristics and outcomes. Importantly, immune dysregulation has been the common driving force between MDS and AIDs pathogenesis.
Conclusion: DAH is a severe disorder, with nonspecific presentation, caused by a wide spectrum of disease, as demonstrated by this series. Diagnosis requires high clinical suspicion and a thorough work-up.
Conclusion This nationwide study demonstrates the efficacy of steroids for SIAD-associated MDSs but a high frequency of steroid dependence. The response to biologics seems low, but rituximab and azacytidine seem promising.
Abstract Thalidomide and its derivatives are immunomodulatory drugs (IMiDs) known for their sedative, teratogenic, anti-angiogenic, and anti-inflammatory properties. Commonly used in the treatment of cancers such as multiple myeloma and myelodysplastic syndrome (MDS), IMiDs have also been used in the treatment of an inflammatory skin pathology associated with Hansen's disease/leprosy. They have also shown promise in the treatment of autoimmune disorders including systemic lupus erythmatosus (SLE) and inflammatory bowel disease (IBD). Recent structural and experimental observations have revolutionized our understan...
(Mayo Clinic) Mayo Clinic researchers have found that azathioprine, a drug commonly used to treat autoimmune disease, may increase the risk of myeloid neoplasms. Myeloid neoplasms include a spectrum of potentially life-threatening bone marrow disorders, such as myelodysplastic syndromes and acute myeloid leukemia. The results are published in JAMA Oncology.
PHOENIX ? Mayo Clinic researchers have found that azathioprine, a drug commonly used to treat autoimmune disease, may increase the risk of myeloid neoplasms. Myeloid neoplasms include a spectrum of potentially life-threatening bone marrow disorders, such as myelodysplastic syndromes and acute myeloid leukemia. The results are published in JAMA Oncology. Researchers analyzed more than 40,000 [...]