BACKGROUND: Myelodysplastic syndromes (MDS) are a collection of clonal neoplastic bone marrow disorders characterized by ineffective hematopoiesis and cytopenias. A comprehensive assessment of the disease burden is lacking.

OBJECTIVES: This review aimed to systematically identify, review, and synthesize the evidence for epidemiologic and humanistic burden of the disease as part of assessing the burden of MDS.

METHODS: MEDLINE and EMBASE were searched from inception to July 13, 2021, following the practices recommended by the PRISMA guidelines. The search strategies included the terms on epidemiology (ie, prevalence and incidence) or health-related quality of life (HRQOL), combined with the disease terms. Two reviewers screened and reviewed all articles independently and in duplicate.

RESULTS: 7539 records were identified in the initial search. The annual age-standardized incidence rate (ASIR, per 100,000) of MDS was reported in 18 unique studies from 12 countries. The median of the ASIR in adults was 3.70 (range, 0.98-11.52). The crude prevalence rate per 100,000 was reported in 5 studies from 4 countries, ranging from 6.2 to 155. Among the 13 studies that reported HRQOL (n=11 to 1985) using European Organisation for Research and Treatment of Cancer (EORTC) QLQ-C30 global score (scale 0-100; higher score for better health for all instruments), mean score was 55.5. Five studies reported Functional Assessment of Cancer Therapy-Anemia (FACT-AN) total score (scale 0-188), with a mean of 121.6 (range, 111-132). Five studies used the EQ-5D-3L or EORTC CLU-C10D to measure health utilities that ranged from 0.73 to 0.87 (scale 0-1). There were few longitudinal studies on HRQOL for MDS, most of which did not find significant improvement in HRQOL over time.

CONCLUSIONS: The prevalence and incidence of MDS varied substantially across countries. The impact of MDS on the QOL of patients was reported as moderate to severe, depending on disease severity and the instrument used.