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. 2025 Sep 22;5(9):e0005197.
doi: 10.1371/journal.pgph.0005197. eCollection 2025.

Burden of hemoglobinopathies and hemolytic anemias in the World Health Organization African region, 2000-2021: Findings from the Global Burden of Disease 2021 study

Affiliations

Burden of hemoglobinopathies and hemolytic anemias in the World Health Organization African region, 2000-2021: Findings from the Global Burden of Disease 2021 study

GBD 2021 Hemoglobinopathies and Hemolytic Anemias Collaborators et al. PLOS Glob Public Health. .

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.

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Conflict of interest statement

L M M Tshilolo reports grants or contracts from Research Evaluation and Commercialization Hubs (REACH)/ National Heart, Lung, and Blood Institute (NIHLB) for a study on the use of hydroxyurea in Sub-Saharan African countries, and the SickleInAfrica consortium; consulting fees from Novo Nordisk and Novartis; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Novo Nordisk for the AS FacultyHeroes Course; support for attending meetings and/or travel from Novo Nordisk and WHO AFRO; leadership or fiduciary roles in other board, society, committee or advocacy groups, paid or unpaid, with REDAC as President, and the SickleInAfrica consortium as Co-chair; other financial or non-financial interests with the Belgian Development Cooperation (DGD) Association for Cultural, Technical and Educational Cooperation (ACTEC) via an education project for bio-technicians in the Democratic Republic of the Congo; all outside the submitted work.

Figures

Fig 1
Fig 1. HHA age-standardized death rates: WHO Africa vs. global and by subtype (2001–2021).
A, Comparison of WHO-African region to global age-standardised death rate due to HHA from 2000 to 2021. B, WHO Africa Region Age-standardised death rate by HHA subtype, 2000–2021. The global age-standardized death rate is represented as a line graph for comparison.
Fig 2
Fig 2. Regional age-standardized mortality rate attributed to HHA in the WHO Africa region from 2000–2021.
Fig 3
Fig 3. Age-standardized HHA death and DALY rates by sex, 2000–2021.
A, Age-standardized cause of death rate (per 100,000 population) attributed to hemoglobinopathies from 2000–2021 stratified by sex. B, Age-standardized DALY rates (per 100,000 population) attributed to hemoglobinopathies from 2000–2021 stratified by sex.
Fig 4
Fig 4. Male-to-female ratios for HHA deaths and birth incidence in the WHO African Region, 2000–2021.
A, Male-to-female cause of death rate ratio by Haemoglobinopathies type in WHO Africa Region, from 2000–2021. B, Male-to-female birth incidence rate ratio by hemoglobinopathies type in WHO Africa Region, from 2000 to 2021.
Fig 5
Fig 5. Age-stratified cause-specific mortality rates for HHA in the WHO African Region, 2000–2021.
A, Cause-specific mortality rate (per 100,000 populations) attributed to thalassemias from 2000–2021, stratified by age. B, Cause-specific mortality rate (per 100,000 population) attributed to sickle cell disorders form 2000–2021, stratified by age. C, Cause-specific mortality rate (per 100,000 population) attributed to G6PD deficiency from 2000–221, stratified by age. D, Cause-specific mortality rate (per 100,000 population) attributed to other hemoglobinopathies and hemolytic anemias from 2000–2021, stratified by age.

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