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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]
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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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“In 1994, the median age at death for males and females was 42 and 48 years, respectively.(8) In 2019, the median age at death was 48.”
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“Added evidence in support of an inflammatory systemic milieu in sickle cell disease is provided by elevated leukocytic counts which occur in these patients, and which correlate with their propensity to vaso-occlusive complications and overall mortality in sickle cell disease.(48) These findings, in conjunction with el…”
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“This approach is preferable to (1) customary rote prescription of the same drug, dose, and interval for each event; (2) as-needed dosing, which permits pain to recur before treatment is provided; or (3) prejudicial exclusive use or avoidance of a particular analgesic.”
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“[10–13].”
Self-citationFatal sickle cell crisis after granulocyte colony-stimulating factor administration
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Structure-Function Relations of Human Hemoglobins
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“Leukocytosis itself is a risk factor for increased mortality (24).”
BackgroundParseval, L. M., Verhelle, D., Glezer, E., Jensen-Pergakes, K., Ferguson, G. D., Corral, L. G., Morris, C. L., Muller, G. W., Brady, H. A., & Chan, K. W. H. (2008) · Journal of Clinical Investigation · doi:10.1172/jci32322 · Cited by 184
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Therapeutic strategies for sickle cell disease: towards a multi-agent approach
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Pulmonary Complications of Sickle Cell Disease
Miller, A. C., & Gladwin, M. T. (2012) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201111-2082ci · Cited by 188
Mammalian gene targeting with designed zinc finger nucleases
Porteus, M. H. (2005) · Molecular Therapy · doi:10.1016/j.ymthe.2005.08.003 · Cited by 180
“dtd" [ ]> Introduction Sickle cell disease is the prototypical monogenic disease in which a single point mutation causes a disease characterized by early mortality and lifelong morbidit”
Thrombophilia in sickle cell disease: the red cell connection
Setty, Y. B. N., Rao, A. K., & Stuart, M. J. (2001) · Blood · doi:10.1182/blood.v98.12.3228 · Cited by 180
Panepinto, J. A., Walters, M. C., Carreras, J., Marsh, J. C. W., Bredeson, C., Gale, R. P., Hale, G. A., Horan, J., Hows, J., Klein, J. P., et al. (2007) · British Journal of Haematology · doi:10.1111/j.1365-2141.2007.06592.x · Cited by 177
“The most frequent adverse events are painful vaso-occlusive crises (VOC) that often require hospitalisation (Platt et al, 1991, 1994).”
BackgroundSickle Cell Disease: New Opportunities and Challenges in Africa
Makani, J., Ofori-Acquah, S. F., Nnodu, O. E., Wonkam, A., & Ohene‐Frempong, K. (2013) · The Scientific World JOURNAL · doi:10.1155/2013/193252 · Cited by 180
“Higher expression of HbF in adulthood ameliorates morbidity and mortality in SCD [56, 95].”
BackgroundWilkie, D. J., Molokie, R. E., Boyd-Seal, D., Suarez, M. L., Kim, Y. O., Zong, S., Wittert, H., Zhao, Z., Saunthararajah, Y., & Wang, Z. J. (2010) · Journal of the National Medical Association · doi:10.1016/s0027-9684(15)30471-5 · Cited by 177
Antagonistic pleiotropy as a widespread mechanism for the maintenance of polymorphic disease alleles
Carter, A. J. R., & Nguyen, A. Q. (2011) · BMC Medical Genetics · doi:10.1186/1471-2350-12-160 · Cited by 176
“Although life-expectancy of individuals with the homozygous Hb-S genotype has increased in recent years in developed nations (median lifespan of 42-48 in the US) [26], for most of human history (and even now in undeveloped nations) homozygous individuals had very short expected life expectancies (I.”
BackgroundTrends in Sickle Cell Disease–Related Mortality in the United States, 1979 to 2017
Payne, A. B., Mehal, J. M., Chapman, C., Haberling, D. L., Richardson, L. C., Bean, C. J., & Hooper, W. C. (2020) · Annals of Emergency Medicine · doi:10.1016/j.annemergmed.2020.08.009 · Cited by 174
International Encyclopedia of Rehabilitation
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Reawakening fetal hemoglobin: prospects for new therapies for the β-globin disorders
Bauer, D. E., Kamran, S. C., & Orkin, S. H. (2012) · Blood · doi:10.1182/blood-2012-06-292078 · Cited by 177
Sickle cell disease in Africa: burden and research priorities
Makani, J., Williams, T. N., & Marsh, K. (2007) · Annals of Tropical Medicine and Parasitology · doi:10.1179/136485907x154638 · Cited by 172
“…has shown that the highest levels of SCD-related mortality occur in children aged between 6 months and 3 years, the commonest causes of death being acute chest syndrome (ACS), acute splenic sequestration and pneumococcal septicaemia (Rogers et al., 1978; Leikin et al., 1989; Platt et al., 1994).”
BackgroundInfluentialAtherogenesis and iron: from epidemiology to cellular level
Vinchi, F., Muckenthaler, M. U., da Silva, M. C., Balla, G. J., Balla, J., & Jeney, V. (2014) · Frontiers in Pharmacology · doi:10.3389/fphar.2014.00094 · Cited by 176
2-deoxy 5-azacytidine and fetal hemoglobin induction in sickle cell anemia
Koshy, M., Dorn, L., Bressler, L. R., Molokie, R. E., Lavelle, D., Talischy, N., Hoffman, R., van Overveld, W., & DeSimone, J. (2000) · Blood · doi:10.1182/blood.v96.7.2379 · Cited by 170
Hydroxyurea (hydroxycarbamide) for sickle cell disease
Nevitt, S. J., Jones, A. P., & Howard, J. (2017) · Cochrane Database of Systematic Reviews · doi:10.1002/14651858.cd002202.pub2 · Cited by 170
Hebbel, R. P. (2010) · American Journal of Hematology · doi:10.1002/ajh.21952 · Cited by 171
“However, a closer examination reveals that mortality actually correlated positively (not inversely) with Hct for the subjects over 20 years of age [156].”
BackgroundResultPulmonary Hypertension and Cor Pulmonale during Severe Acute Chest Syndrome in Sickle Cell Disease
Dessap, A. M., Rusel, L., Habibi, A., Nzouakou, R., Roudot‐Thoraval, F., Adnot, S., Godeau, B., Galactéros, F., Brun‐Buisson, C., Brochard, L. J., et al. (2008) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200710-1606oc · Cited by 168
“On the basis of our findings, pulmonary hypertension and cor pulmonale may explain the high rate of death in patients with ACS complicating SCD ( 4 , 6). In our study, eight of the nine deaths occurred in patients who had TRV values of 3 m/second or greater during at least one severe ACS episode.”
BackgroundInfluentialGastrointestinal and Hepatic Complications of Sickle Cell Disease
Ebert, E. C., Nagar, M., & Hagspiel, K. D. (2010) · Clinical Gastroenterology and Hepatology · doi:10.1016/j.cgh.2010.02.016 · Cited by 168
Hemoglobin sickle cell disease complications: a clinical study of 179 cases
Lionnet, F., Hammoudi, N., Stojanovic, K. S., Avellino, V., Grateau, G., Girot, R., & Haymann, J. (2012) · Haematologica · doi:10.3324/haematol.2011.055202 · Cited by 168
Placenta growth factor activates monocytes and correlates with sickle cell disease severity
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Hydroxyurea and sickle cell anemia: effect on quality of life
Ballas, S. K., Barton, F. B., Waclawiw, M. A., Swerdlow, P. S., Eckman, J. R., Pegelow, C. H., Koshy, M., BARTON, B. A., & Bonds, D. R. (2006) · Health and Quality of Life Outcomes · doi:10.1186/1477-7525-4-59 · Cited by 167
“In the Cooperative Study of Sickle Cell Disease (CSSCD), an analysis of painful crises in 3,578 patients ranging in age from newborns to age 66 [5-7] conducted from 1982–1995, the average crisis rate was 0.”
BackgroundNovelli, E. M., & Gladwin, M. T. (2015) · CHEST Journal · doi:10.1016/j.chest.2015.12.016 · Cited by 166
“It has been hypothesized that sudden death during VOC may be due to fat embolization that has not yet resulted in MOFS.(25) Acute respiratory distress syndrome physiology can complicate MOFS in one-quarter of cases and require intubation and mechanical ventilation with standard lung-protective strategies (ie, low tida…”
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The intriguing contribution of white blood cells to sickle cell disease – a red cell disorder
Okpala, I. E. (2003) · Blood Reviews · doi:10.1016/s0268-960x(03)00037-7 · Cited by 166
Understanding Sickle cell disease: Causes, symptoms, and treatment options
Elendu, C., Amaechi, D. C., Alakwe-Ojimba, C. E., Elendu, T. C., Elendu, R. C., Ayabazu, C. P., Aina, T. O., Aborisade, O., & Adenikinju, J. S. (2023) · Medicine · doi:10.1097/md.0000000000035237 · Cited by 189
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Cardiovascular complications and risk of death in sickle-cell disease
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Erythropoiesis: insights into pathophysiology and treatments in 2017
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Gene Therapy of the β-Hemoglobinopathies by Lentiviral Transfer of the β A(T87Q) - Globin Gene
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MYH9 and APOL1 are both associated with sickle cell disease nephropathy
Ashley‐Koch, A. E., Okocha, E. C., Garrett, M. E., Soldano, K. L., Castro, L. M. D., Jonassaint, J., Orringer, E. P., Eckman, J. R., & Telen, M. J. (2011) · British Journal of Haematology · doi:10.1111/j.1365-2141.2011.08832.x · Cited by 171
“Nephropathy is a major risk factor for early mortality in SCD (Platt et al, 1994).”
BackgroundInfluentialA network model to predict the risk of death in sickle cell disease
Sebastiani, P., Nolan, V. G., Baldwin, C. T., Abad‐Grau, M. M., Wang, L., Adewoye, A. H., McMahon, L. C., Farrer, L. A., Taylor, J. G., Kato, G. J., et al. (2007) · Blood · doi:10.1182/blood-2007-04-084921 · Cited by 163
“Correspondence: Martin H. Steinberg, Department of Medicine, Boston University School of Medicine, Rm E248, 88 East Newton Street, Boston, MA 02118; e-mail: mhsteinb@bu.edu.”
BackgroundInfluentialSelf-citationThe effect of hydroxcarbamide therapy on survival of children with sickle cell disease
Lobo, C. L. D. C., Pinto, J. F. D. C., do Nascimento, E. M., de Moura, P. G., Cardoso, G. P., & Hankins, J. S. (2013) · British Journal of Haematology · doi:10.1111/bjh.12323 · Cited by 163
“…from SCD complications in children include acute chest syndrome (ACS), sepsis, splenic sequestration, stroke, aplastic crisis from parvovirus B19 infection, and, less commonly, organ failure (Leikin et al, 1989; Platt et al, 1994; Powars et al, 2005; Darbari et al, 2006; Brousse et al, 2012).”
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“In the natural history of SCD, several cohort studies have estimated that the chance of developing a clinical stroke by the age of 18 to 20 years is 11%.(1,9) This estimate however does not reflect the implementation of transcranial Doppler (TCD) screening tests that resulted from a stroke prevention trial in sickle c…”
Background
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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