Background: Autoimmune disorders (ADs) are prevalent among patients with myelodysplastic syndrome (MDS), yet their impact on MDS outcomes, including overall survival (OS), mortality, and transformation to acute myeloid leukemia (AML), is not well defined. Methods: We conducted a systematic review of articles published up to April 2024, sourced from PubMed, Web of Science, Embase, and Google Scholar, focusing on the influence of ADs on survival and AML transformation rates in MDS patients. The methodological quality of each study was assessed using the Newcastle Ottawa Scale. Results: From 8 studies that met the inclusion criteria, ADs were present in 17.5% (3074/17,481) of MDS patients. Data analysis indicated mortality rates ranging from 15.3% to 67% in MDS patients with ADs and 12% to 69% in those without. The rate of AML transformation varied from 0% to 23% in patients with ADs compared to 4% to 30% in those without. Conclusions: The influence of ADs on survival and AML transformation in MDS patients appears variable. This systematic review highlights the need for further large-scale prospective studies to clarify the relationship between ADs and MDS outcomes.
