Abstract
Hemoglobinopathies and hemolytic anemias (HHA) are genetic blood disorders associated with diverse clinical complications, affecting an estimated 2.1 billion people worldwide. The World Health Organization (WHO) African Region accounts for approximately 425.8 million individuals, or 20% of the global HHA prevalence, yet comprehensive assessments of this burden have been lacking. We present the first systematic analysis of HHA burden in the WHO African Region from 2000-2021 using data from the Global Burden of Disease (GBD) 2021 study. We estimated regional, sex-, and age-specific rates (per 100,000 population) of mortality, incidence at birth, and years lived with disability (YLDs) in five-year intervals. Mortality estimates were generated using the Cause of Death Ensemble model (CODEm), supplemented with spatiotemporal Gaussian process regression. Incidence at birth was estimated using DisMod-MR 2.1, a Bayesian meta-regression tool, while YLDs were calculated by multiplying prevalence by disability weights reflecting severity and duration. Between 2000 and 2021, the WHO African Region experienced persistently higher age-standardized death rates from HHA compared to global levels, although regional mortality declined over the period. Sickle cell disorder (SCD) was the predominant contributor, with the highest mortality [3.68 deaths (95% UI 2.04-6.29) per 100,000] and disability burden [41.08 YLDs (95% UI 26.09-58.61)], while thalassemias contributed the least. Disability-adjusted life years (DALYs) were concentrated in western sub-Saharan Africa, accounting for 71.3% of the regional burden. Age-specific estimates revealed that children under five years faced a disproportionate share of mortality and disability. Despite overall declines in mortality, the WHO African Region continues to bear a disproportionate global burden of HHA, particularly affecting young children. These findings underscore the urgent need for strengthened newborn screening, early treatment, and health system interventions to reduce preventable deaths and disability.