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Mortality in sickle cell disease. Life expectancy and risk factors for early death
Open accessAbstract
BACKGROUND: Information on life expectancy and risk factors for early death among patients with sickle cell disease (sickle cell anemia, sickle cell-hemoglobin C disease, and the sickle cell-beta-thalassemias) is needed to counsel patients, target therapy, and design clinical trials. METHODS: We followed 3764 patients who ranged from birth to 66 years of age at enrollment to determine the life expectancy and calculate the median age at death. In addition, we investigated the circumstances of death for all 209 adult patients who died during the study, and used proportional-hazards regression analysis to identify risk factors for early death among 964 adults with sickle cell anemia who were followed for at least two years. RESULTS: Among children and adults with sickle cell anemia (homozygous for sickle hemoglobin), the median age at death was 42 years for males and 48 years for females. Among those with sickle cell-hemoglobin C disease, the median age at death was 60 years for males and 68 years for females. Among adults with sickle cell disease, 18 percent of the deaths occurred in patients with overt organ failure, predominantly renal. Thirty-three percent were clinically free of organ failure but died during an acute sickle crisis (78 percent had pain, the chest syndrome, or both; 22 percent had stroke). Modeling revealed that in patients with sickle cell anemia, the acute chest syndrome, renal failure, seizures, a base-line white-cell count above 15,000 cells per cubic millimeter, and a low level of fetal hemoglobin were associated with an increased risk of early death. CONCLUSIONS: Fifty percent of patients with sickle cell anemia survived beyond the fifth decade. A large proportion of those who died had no overt chronic organ failure but died during an acute episode of pain, chest syndrome, or stroke. Early mortality was highest among patients whose disease was symptomatic. A high level of fetal hemoglobin predicted improved survival and is probably a reliable childhood forecaster of adult life expectancy.
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“Elevated levels of fetal hemoglobin (consisting of two alpha and two gamma chains) are associated with improved morbidity and mortality in patients with TDT and SCD.[20][21][22]”
From CRISPR-Cas9 Gene Editing for Sickle Cell Disease and β-Thalassemia (2021) · cites this paper as [21]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
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“the presence of chronic kidney disease is an independent predictor of pulmonary vascular disease and early mortality in adults with SCD (68, 202).”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [68]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
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“In 3764 patients in the pre-hydroxyurea era, HbF .8.6% was associated with improved survival.60”
From Fetal hemoglobin in sickle cell anemia (2020) · cites this paper as [60]
Experimental — accuracy not yet measuredIn the citing paper
“Patients affected by sickle cell anemia and β-thalassemia experience a milder course of the disease if they express elevated levels of fetal hemoglobin in adulthood (Platt et al., 1994; Weatherall, 2001).”
From Reactivation of developmentally silenced globin genes by forced chromatin looping (2014) · cites this paper as Platt et al., 1994
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Citations
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Alsultan, A., Solovieff, N., Aleem, A., AlGahtani, F. H., Al‐Shehri, A., Elfaki Osman, M., Kurban, K., Bahakim, H., Kareem Al‐Momen, A., Baldwin, C. T., et al. (2011) · American Journal of Hematology · doi:10.1002/ajh.22032 · Cited by 32
Self-citationRisk factor analysis of cerebral white matter hyperintensities in children with sickle cell disease
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“HbF is one of the most important known modifiers of disease severity in SCD, predicting mortality, pain rates, dactylitis and acute chest syndrome (Platt et al, 1994, 1991; Bhatnagar et al, 2013; Bailey et al, 1992; Castro et al, 1994).”
BackgroundSingle-session Biofeedback-assisted Relaxation Training in Children With Sickle Cell Disease
Myrvik, M. P., Campbell, A. D., & Butcher, J. L. (2012) · Journal of Pediatric Hematology/Oncology · doi:10.1097/mph.0b013e318253f0ba · Cited by 29
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“In individuals with HbSS, the level of HbF is variable but may reach 20%, with high levels of HbF associated with amelioration of disease severity [7].”
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“Many studies have shown %HbF levels to be associated with clinical and/or biological protective effects.(2,36) The association between %HbF and the presence and burden of WMCs described here is highly consistent with the known antipolymerization dose-response effect of %HbF.”
ResultAeroallergen sensitization predicts acute chest syndrome in children with sickle cell anaemia
Willen, S. M., Rodeghier, M., Strunk, R. C., Bacharier, L. B., Rosen, C. L., Kirkham, F. J., DeBaun, M. R., & Cohen, R. T. (2018) · British Journal of Haematology · doi:10.1111/bjh.15076 · Cited by 20
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“Fetal hemoglobin (HbF) is the major modulator of the phenotype of sickle cell anemia and increased concentrations can reduce disease severity.(1, 2) HbF levels are controlled by”
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“[35, 36] In the Middle East and in India the HbS gene is often on the AI HBB haplotype.”
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Boulassel, M.-R., Al-Badi, A., Elshinawy, M., Al-Hinai, J., Al-Saadoon, M., Al-Qarni, Z., Khan, H., Qureshi, R. N., & Wali, Y. (2019) · Quality of Life Research · doi:10.1007/s11136-018-2031-0 · Cited by 14
Chronic administration of hydroxyurea (HU) benefits caucasian patients with sickle-beta thalassemia
Di Maggio, R., Hsieh, M., Zhao, X., Calvaruso, G., Rigano, P., Renda, D., Tisdale, J., & Maggio, A. (2018) · International Journal of Molecular Sciences · doi:10.3390/ijms19030681 · Cited by 12
“While it was prescribed from 50 to 75% by US hematologists [26,27].”
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Cerebrovascular accidents in sickle cell disease: rates and risk factors.
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Metadata
- Authors
- Platt, O. S., Brambilla, D. J., Rosse, W. F., Milner, P. F., Castro, O., Steinberg, M. H., & Klug, P. P.
- Affiliations
- Boston Children's Hospital (US)
- New England Research Institutes (US)
- Duke University (US)
- Augusta University (US)
- Howard University (US)
- University of Mississippi Medical Center (US)
- Texas Tech University (US)
- Venue
- New England Journal of Medicine
- Publisher
- Massachusetts Medical Society
- Published
- 1994-06-09
- Volume
- 330
- Issue
- 23
- Pages
- 1639-1644
- ISSN
- 0028-4793 (ISSN-L)
- 1533-4406
- OpenAlex
- W2334263927
- Semantic Scholar
- 6bcc7a4d436214c41a4d128f9d221e950eb4962b
- Cited by
- 3,320
- References
- 19
- Citations by year
- 2026: 83 · 2025: 104 · 2024: 123 · 2023: 135 · 2022: 128 · 2021: 167 · 2020: 147 · 2019: 168 · 2018: 140 · 2017: 159 · 2016: 173 · 2015: 165 · 2014: 154 · 2013: 139 · 2012: 115
- FWCI
- 24.43
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- New England Journal of Medicine (publishedVersion)
- PubMed (publishedVersion)
- Digital Howard (Howard University) (submittedVersion)
- Topics
- Health Sciences › Medicine › Genetics (clinical) › Hemoglobinopathies and Related Disorders
- Health Sciences › Medicine › Public Health, Environmental and Occupational Health › Malaria Research and Control
- Health Sciences › Medicine › Hematology › Blood groups and transfusion
- Keywords
- sickle cell disease, sickle cell-hemoglobin C disease, sickle beta-thalassemia, life expectancy, mortality risk factors, acute chest syndrome, vaso-occlusive crisis, stroke, renal failure, seizures, fetal hemoglobin, white blood cell count, organ failure
- MeSH terms
- Adult, Aged, Anemia, Sickle Cell, Cause of Death, Child, Female, Hemoglobin SC Disease, Humans, Life Expectancy, Male, Middle Aged, Probability, Regression Analysis, Risk Factors, Survival Analysis, beta-Thalassemia
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