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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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“The Cooperative Study of Sickle Cell Disease (CSSCD), a multicenter natural history study of SCD conducted between 1978 and 1988, reported median ages at death during the study period of 42 and 48 years, respectively, for male and female HbSS patients, and median ages at death of 60 and 68 years, respectively, for mal…”
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BackgroundResultInfluentialComparisons of High Versus Low Emergency Department Utilizers in Sickle Cell Disease
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High one year mortality in adults with sickle cell disease and end‐stage renal disease
McClellan, A. C., Luthi, J., Lynch, J. R., Soucie, J. M., Kulkarni, R., Guasch, A., Huff, E. D., Gilbertson, D. T., McClellan, W. M., & DeBaun, M. R. (2012) · British Journal of Haematology · doi:10.1111/bjh.12024 · Cited by 133
“Prior to recent innovations in treatment protocols that have increased the life expectancy for this population, children born with SCD often died during early adolescence (Platt et al , 1994).”
First Unaffected Pregnancy Using Preimplantation Genetic Diagnosis for Sickle Cell Anemia
Xu, K. (1999) · JAMA · doi:10.1001/jama.281.18.1701 · Cited by 135
“Life expectancy for persons with sickle cell disease varies and there is an age-related pattern in mortality rates: a peak in patients younger than 5 years, with a gradual increase starting in late adolescence.(5) Progress in the treatment of sickle cell disease has been slow.”
BackgroundRole of free radicals in the pathogenesis of acute chest syndrome in sickle cell disease
Klings, E. S., & Farber, H. W. (2001) · Respiratory Research · doi:10.1186/rr70 · Cited by 132
“Peripheral WBC counts are elevated in VOC and ACS, and WBC counts greater than 15,000 are associated with increased mortality [26,27].”
Glomerular hyperfiltration and albuminuria in children with sickle cell anemia
Aygün, B., Mortier, N. A., Smeltzer, M. P., Hankins, J. S., & Ware, R. E. (2011) · Pediatric Nephrology · doi:10.1007/s00467-011-1857-2 · Cited by 132
“The development of focal segmental glomerulosclerosis or membranoproliferative glomerulonephritis, hyperfiltration, albuminuria and iron deposition may contribute to diminished glomerular filtration and result in end stage renal disease that affects many young adults with sickle cell anemia (SCA) [1, 2 ]. The pathophy…”
BackgroundSickle‐cell haemoglobin polymerization: is it the primary pathogenic event of sickle‐cell anaemia?
Vekilov, P. G. (2007) · British Journal of Haematology · doi:10.1111/j.1365-2141.2007.06794.x · Cited by 132
“…clinical manifestations of sickle cell anaemia are dramatically different in patients with identical concentrations of HbS in their red blood cells (Amin et al, 1991; el-Hazmi, 1992a; Platt et al, 1994; Bridges et al, 1996), with no identified factors that could result in HbS solubility variations.”
BackgroundProphylactic antibiotics for preventing pneumococcal infection in children with sickle cell disease
Rankine‐Mullings, A., & Owusu‐Ofori, S. (2017) · Cochrane Database of Systematic Reviews · doi:10.1002/14651858.cd003427.pub4 · Cited by 132
Pathophysiology of sickle cell disease: Role of cellular and genetic modifiers
Steinberg, M. H., & Rodgers, G. P. (2001) · Seminars in Hematology · doi:10.1016/s0037-1963(01)90023-x · Cited by 131
Self-citationGranulocytosis causing sickle-cell crisis
Abboud, M. R., Laver, J. H., & Blau, C. A. (1998) · The Lancet · doi:10.1016/s0140-6736(05)60614-9 · Cited by 131
Activated monocytes and platelet-monocyte aggregates in patients with sickle cell disease*
WUN, T., CORDOBA, M., RANGASWAMI, A., CHEUNG, A. W., & PAGLIERONI, T. (2002) · Clinical and Laboratory Haematology · doi:10.1046/j.1365-2257.2002.t01-1-00433.x · Cited by 130
Activated monocytes and platelet-monocyte aggregates in patients with sickle cell disease*
Wun, T., CORDOBA, M., Rangaswami, A. A., Cheung, A., & Paglieroni, T. G. (2002) · Clinical & Laboratory Haematology · doi:10.1046/j.1365-2257.2002.00433.x · Cited by 130
Byun, H., Hillman, T. R., Higgins, J. M., Diez-Silva, M., Peng, Z., Dao, M., Dasari, R. R., Suresh, S., & Park, Y. (2012) · Acta Biomaterialia · doi:10.1016/j.actbio.2012.07.011 · Cited by 128
“As a result of these complications and the limited choices for medical treatments, life expectancy for SCD patients is short; only 50% of patients with SCD survive beyond their fifth decade [2].”
BackgroundKrishnamurti, L., Kharbanda, S., Biernacki, M. A., Zhang, W., Baker, K. S., Wagner, J. E., & Wu, C. J. (2008) · Transplantation and Cellular Therapy · doi:10.1016/j.bbmt.2008.08.016 · Cited by 128
Haemoglobinopathy and sickle cell disease
Wilson, M., Forsyth, P. D., & Whiteside, J. (2009) · Continuing Education in Anaesthesia Critical Care & Pain · doi:10.1093/bjaceaccp/mkp038 · Cited by 128
Díaz, E., García, L., Hernández, M., Palacio, L., Ruiz, D., Velandia, N., Villavicencio, J., & Moreno, F. (2012) · Europe PMC (PubMed Central) · Cited by 126
Walters, M. C., Castro, L. M. D., Sullivan, K. M., Krishnamurti, L., Kamani, N. R., Bredeson, C., Neuberg, D. S., Hassell, K. L., Farnia, S. H., Campbell, A., et al. (2015) · Transplantation and Cellular Therapy · doi:10.1016/j.bbmt.2015.10.017 · Cited by 128
“HCT IN CHILDREN WITH SCD In developing eligibility criteria for HCT in early studies, investigators selected clinical features of SCD that carry a high burden of ongoing supportive care with a risk of cumulative organ injury and an association with early mortality [11].”
Minireview: Genetic basis of heterogeneity and severity in sickle cell disease
Habara, A. H., & Steinberg, M. H. (2016) · Experimental Biology and Medicine · doi:10.1177/1535370216636726 · Cited by 133
“6%.(22) The tricuspid regurgitant velocity predicted near-term death.”
BackgroundSelf-citationAnxiety and depression in children and adolescents with sickle cell disease
Benton, T. D., Ifeagwu, J. A., & Smith‐Whitley, K. (2007) · Current Psychiatry Reports · doi:10.1007/s11920-007-0080-0 · Cited by 127
Gender and haplotype effects upon hematological manifestations of adult sickle cell anemia
Steinberg, M. H., Hsu, H., Nagel, R. L., Milner, P., Adams, J. G., Benjamin, L. J., Fryd, S., GILLETTE, P. N., Gilman, J. G., Josifovska, O., et al. (1995) · American Journal of Hematology · doi:10.1002/ajh.2830480307 · Cited by 126
Self-citationThe nephropathy of sickle cell trait and sickle cell disease
Ataga, K. I., Saraf, S. L., & Derebail, V. K. (2022) · Nature Reviews Nephrology · doi:10.1038/s41581-022-00540-9 · Cited by 125
Machado, R. F., & Gladwin, M. T. (2005) · British Journal of Haematology · doi:10.1111/j.1365-2141.2005.05432.x · Cited by 123
“According to the Cooperative Study of Sickle Cell Disease (CSSCD), a prospective multicentre study of 3764 patients, over 20% of adults probably had fatal pulmonary complications of SCD (Platt et al, 1994).”
BackgroundFetal hemoglobin in sickle cell anemia: genetic determinants of response to hydroxyurea
Ma, Q., Wyszynski, D. F., Farrell, J. J., Kutlar, A., Farrer, L. A., Baldwin, C. T., & Steinberg, M. H. (2007) · The Pharmacogenomics Journal · doi:10.1038/sj.tpj.6500433 · Cited by 125
Self-citationKumar, A. A., Patton, M. R., Hennek, J. W., Lee, S. Y. R., D’Alesio-Spina, G., Yang, X., Kanter, J., Shevkoplyas, S. S., Brugnara, C., & Whitesides, G. M. (2014) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1414739111 · Cited by 124
Sickle cell detection using a smartphone
Knowlton, S. M., Şencan, İ., Aytar, Y., Khoory, J. A., Heeney, M. M., Ghiran, I. C., & Taşoğlu, S. (2015) · Scientific Reports · doi:10.1038/srep15022 · Cited by 124
Guidelines on red cell transfusion in sickle cell disease Part II : indications for transfusion
Davis, B. A., Allard, S., Qureshi, A., Porter, J. B., Pancham, S., Win, N., Cho, G., Ryan, K. E., & the British Society for Haematology (2016) · British Journal of Haematology · doi:10.1111/bjh.14383 · Cited by 124
“Acute chest syndrome is a marker of SCD severity, being a risk factor for early death in SS patients >20 years (Castro et al, 1994; Platt et al, 1994) and 44% of patients will have recurrent episodes (Castro et al, 1994).”
SYSTEMIC FAT EMBOLISM AND PULMONARY HYPERTENSION IN SICKLE CELL DISEASE
Castro, O. (1996) · Hematology/Oncology Clinics of North America · doi:10.1016/s0889-8588(05)70401-9 · Cited by 122
Self-citationTsitsikas, D. A., Gallinella, G., Patel, S. R., Seligman, H., Greaves, P., & Amos, R. J. (2014) · Blood Reviews · doi:10.1016/j.blre.2013.12.002 · Cited by 123
“The authors pointed out the uncertainty around the cause of these deaths but mentioned FES as a possibility [18].”
BackgroundSickle Red Cell Microrheology and Sickle Blood Rheology
Ballas, S. K., & Mohandas, N. (2004) · Microcirculation · doi:10.1080/10739680490279410 · Cited by 123
“This includes the finding of moderate elevation of WBC counts of many patients with sickle cell disease in the steady state owing to functional asplenia/autosplenectomy, marked leukocytosis during acute painful crises (129), higher mortality in patients with sickle cell disease who have high WBC counts (109), and coin…”
BackgroundAssociation between hemolysis and albuminuria in adults with sickle cell anemia
Day, T. G., Drašar, E., Fulford, T. J., Sharpe, C. C., & Thein, S. L. (2011) · Haematologica · doi:10.3324/haematol.2011.050336 · Cited by 122
Emergency Department Management of Acute Pain Episodes in Sickle Cell Disease
Tanabe, P. J., Myers, R., Zosel, A. E., Brice, J. H., Ansari, A. H., Evans, J., Martinovich, Z., Todd, K. H., & Paice, J. A. (2007) · Academic Emergency Medicine · doi:10.1111/j.1553-2712.2007.tb01801.x · Cited by 122
Machado, R. F., Mack, A. K., Martyr, S. E., Barnett, C. F., MacArthur, P. H., Sachdev, V., Ernst, I., Hunter, L. A., Coles, W. A., Nichols, J., et al. (2006) · British Journal of Haematology · doi:10.1111/j.1365-2141.2006.06417.x · Cited by 122
“Sudden or unexplained death is a well-described phenomenon in patients with sickle cell disease, both in steady state and during vaso-occlusive crisis, occurring in 12–26% of all deaths in the Co-operative Study of Sickle Cell Disease (Charache, 1994; Platt et al, 1994).”
BackgroundTreadwell, M. J., Telfair, J., Gibson, R. W., Johnson, S., & Osunkwo, I. (2010) · American Journal of Hematology · doi:10.1002/ajh.21880 · Cited by 129
“ized as a lifelong chronic condition, rather than as a disease of childhood [1,2].”
BackgroundMabaera, R., West, R. J., Conine, S. J., Macari, E. R., Boyd, C. D., Engman, C. A., & Lowrey, C. H. (2008) · Experimental Hematology · doi:10.1016/j.exphem.2008.06.014 · Cited by 123
Saraf, S. L., Zhang, X., Kanias, T., Lash, J. P., Molokie, R. E., Oza, B., Lai, C., Rowe, J. H., Gowhari, M., Hassan, J., et al. (2014) · British Journal of Haematology · doi:10.1111/bjh.12690 · Cited by 120
“Chronic kidney disease is a common complication of SCA that is associated with other morbidities and early mortality (Powars et al, 1991, 2005; Platt et al, 1994).”
BackgroundKinetics of sickle cell biorheology and implications for painful vasoocclusive crisis
Du, E., Diez-Silva, M., Kato, G. J., Dao, M., & Suresh, S. (2015) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1424111112 · Cited by 120
“Significant correlations have been shown between pain rates and early death in patients with sickle cell anemia (14) and between early death and several risk factors such as fetal hemoglobin (HbF), hematocrit, and white cell count (15).”
BackgroundDaak, A. A., Ghebremeskel, K., Hassan, Z., Attallah, B., Azan, H. H., Elbashir, M. I., & Crawford, M. A. (2012) · American Journal of Clinical Nutrition · doi:10.3945/ajcn.112.036319 · Cited by 120
Glover, R. E., Ivy, E. D., Orringer, E. P., Maeda, H., & Mason, R. P. (1999) · Molecular Pharmacology · doi:10.1124/mol.55.6.1006 · Cited by 119
“It has been shown that a white blood cell count greater than 15 3 10(9) cells/liter is associated with an increased risk of early death in sickle cell anemia (Platt et al., 1994).”
Tshilolo, L. M., Kafando, É., Sawadogo, M., COTTON, F. J., Vertongen, F., Ferster, A., & Gulbis, B. (2008) · Public Health · doi:10.1016/j.puhe.2007.12.005 · Cited by 118
Recent progress in understanding and manipulating haemoglobin switching for the haemoglobinopathies
Vinjamur, D. S., Bauer, D. E., & Orkin, S. H. (2018) · British Journal of Haematology · doi:10.1111/bjh.15038 · Cited by 114
“The Cooperative Study of SCD showed that patients with HbF levels above the 75th percentile (>8 6% HbF) had extended overall survival (Platt et al, 1994). b-thalassaemia patients with elevated levels of HbF also display less severe disease course (Fessas & Stamatoyannopoulos, 1964; Weatherall,…”
BackgroundStrouse, J. J., Takemoto, C. M., Keefer, J. R., Kato, G. J., & Casella, J. F. (2007) · Pediatric Blood & Cancer · doi:10.1002/pbc.21336 · Cited by 117
“3–9%), but ACS accounts for a significant proportion of mortality in both groups [2,3,5].”
BackgroundSetty, Y. B. N., Kulkarni, S., Rao, A. K., & Stuart, M. J. (2000) · Blood · doi:10.1182/blood.v96.3.1119.015k53_1119_1124 · Cited by 117
Godeau, B., Schaeffer, A., Bachir, D., Fleury-Feith, J., Galacteros, F., Verra, F., Escudier, E., Vaillant, J. N., Brun-Buisson, C., Rahmouni, A., et al. (1996) · American journal of respiratory and critical care medicine · doi:10.1164/ajrccm.153.5.8630622 · Cited by 116
Almeida, C. B., Scheiermann, C., Jang, J.-E., Prophete, C., Costa, F. F., Conran, N., & Frenette, P. S. (2012) · Blood · doi:10.1182/blood-2012-02-409524 · Cited by 120
“), a scavenger of NO that does not affect NOS; or the guanylate cyclase inhibitor, 1H-[1,2,4] oxadiazolo [4,3-a]quinoxalin-”
BackgroundQuinn, C. T. (2016) · Experimental Biology and Medicine · doi:10.1177/1535370216640385 · Cited by 116
“Individuals with HbSC and HbSbþ have survival estimates that approximate the general population.(6) While it is true that SCD is a disease of the blood whose hallmark is the painful vaso-occlusive episode, if one focuses on the hematologic (e.”
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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