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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]
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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 contrast, life-expectancy for SCD patients has improved in western countries and most patients live to their 40s and 50s [6].”
BackgroundMetformin induces FOXO3-dependent fetal hemoglobin production in human primary erythroid cells
Zhang, Y., Paikari, A., Sumazin, P., Summarell, C. C. G., Crosby, J. R., Boerwinkle, E., Weiss, M. J., & Sheehan, V. (2018) · Blood · doi:10.1182/blood-2017-11-814335 · Cited by 67
Acute chest syndrome of sickle cell disease: new light on an old problem
Stuart, M. J., & Setty, Y. B. N. (2001) · Current Opinion in Hematology · doi:10.1097/00062752-200103000-00009 · Cited by 67
Pharmacologic induction of fetal hemoglobin: raising the therapeutic bar in sickle cell disease
Atweh, G. F., & Schechter, A. N. (2001) · Current Opinion in Hematology · doi:10.1097/00062752-200103000-00010 · Cited by 66
Peptide Nucleic Acids as a Tool for Site-Specific Gene Editing
Ricciardi, A. S., Quijano, E., Putman, R., Saltzman, W. M., & Glazer, P. M. (2018) · Molecules · doi:10.3390/molecules23030632 · Cited by 66
“Elevated fetal hemoglobin (HbF), however, reduces the severity of β-thalassemia and sickle cell disease, regardless of the causal mutation [33,34].”
BackgroundAllogeneic stem cell transplantation for sickle cell disease. A study of patients' decisions
van Besien, K., Koshy, M., Anderson-Shaw, L. K., Talischy, N., Dorn, L., Devine, S. M., Yassine, M., & Kodish, E. D. (2001) · Bone Marrow Transplantation · doi:10.1038/sj.bmt.1703208 · Cited by 66
Erythroid adhesion molecules in sickle cell disease: Effect of hydroxyurea
Cartron, J., & Élion, J. (2008) · Transfusion Clinique et Biologique · doi:10.1016/j.tracli.2008.05.001 · Cited by 65
Transcranial Doppler ultrasonography in adults with sickle cell disease
Valadi, N., Silva, G. S., Bowman, L. S., Ramsingh, D. S., Vicari, P., Filho, A. C. F. Q., Massaro, A. R., Kutlar, A., Nichols, F. T., & Adams, R. J. (2006) · Neurology · doi:10.1212/01.wnl.0000230150.39429.8e · Cited by 66
Cohen, R. T., Madadi, A. R., Blinder, M. A., DeBaun, M. R., Strunk, R. C., & Field, J. J. (2011) · American Journal of Hematology · doi:10.1002/ajh.22098 · Cited by 66
“Variables that were analyzed for an association with risk of death included age, SCD phenotype, WBC, hemoglobin, hydroxyurea use, serum creatinine, ACS rate, asthma, and respiratory symptoms [17,18].”
BackgroundNeonatal Screening and the Clinical Outcome in Children with Sickle Cell Disease in Central India
Upadhye, D. S., Jain, D., Trivedi, Y. L., Nadkarni, A. H., Ghosh, K., & Colah, R. B. (2016) · PLoS ONE · doi:10.1371/journal.pone.0147081 · Cited by 66
“Infection particularly pneumococcal sepsis is the leading cause of death in children with SCD [12, 30].”
BackgroundShah, N., Bhor, M., Xie, L., Halloway, R., Arcona, S., Paulose, J., & Yüce, H. (2019) · Health and Quality of Life Outcomes · doi:10.1186/s12955-019-1225-7 · Cited by 66
“Among SCD-related complications, VOC events have long been identified as a higher risk factor for death and the most common cause of hospital admission among SCD patients [15].”
BackgroundThinking beyond sickling to better understand pain in sickle cell disease
Darbari, D. S., Ballas, S. K., & Clauw, D. J. (2014) · European Journal Of Haematology · doi:10.1111/ejh.12340 · Cited by 65
“Higher rates of admission for pain are also associated with early mortality (3, 4).”
BackgroundLazio, M. P., Costello, H. H., Courtney, D. M., Martinovich, Z., Myers, R., Zosel, A. E., & Tanabe, P. J. (2010) · Clinical Journal of Pain · doi:10.1097/ajp.0b013e3181bed10c · Cited by 65
“Acute painful crisis is the most common reason for ED visits, and frequent pain episodes are associated with increased risk of mortality.(9,10) These episodes are characterized as having an abrupt onset and are associated with severe pain.”
BackgroundMechanistic Insights and Characterization of Sickle Cell Disease–Associated Cardiomyopathy
Desai, A. A., Patel, A. R., Ahmad, H., Groth, J., Thiruvoipati, T., Turner, K. M., Yodwut, C., Czobor, P., Artz, N., Machado, R. F., et al. (2014) · Circulation Cardiovascular Imaging · doi:10.1161/circimaging.113.001420 · Cited by 65
Emerging point-of-care technologies for sickle cell disease screening and monitoring
Alapan, Y., Fraiwan, A., Kucukal, E., Hasan, M. N., Ung, R., Kim, M., Odame, I., Little, J. A., & Gurkan, U. A. (2016) · Expert Review of Medical Devices · doi:10.1080/17434440.2016.1254038 · Cited by 66
“The dose needed formaximal clinical benefit, whichmay or may not be the maximum-tolerated dose (MTD) [206], is generally identified within 6–8 months of initiating HU therapy, but should be established and assigned only after the patient tolerates thedose for at least 8weeks [52,58].”
BackgroundSickle cell disease in the older adult
Thein, M. S., Igbineweka, N. E., & Thein, S. L. (2016) · Pathology · doi:10.1016/j.pathol.2016.10.002 · Cited by 71
“To date, no randomised controlled studies are available for the management of osteomyelitis and septic arthritis in both children and adults with SCD.47 Ongoing vasculopathy leads to chronic bone problems such as chronic arthritis, osteonecrosis/avascular necrosis (AVN) of bone, decreased bone growth and decreased bon…”
InfluentialRole of the coagulation system in the pathogenesis of sickle cell disease
Nasimuzzaman, M., & Malik, P. (2019) · Blood Advances · doi:10.1182/bloodadvances.2019000193 · Cited by 65
“The often devastating disease is characterized by RBC sickling; chronic hemolytic anemia; priapism; infections; episodic vaso-occlusion associated with severe pain and inflammation; acute and cumulative organ damage that manifests as stroke, acute chest syndrome, sickle lung disease, pulmonary hypertension, nephropath…”
BackgroundLiesner, Mackie, Cookson, McDonald, Chitolie, Donohoe, S., Evans, Hann, I. M., & Machin (1998) · British Journal of Haematology · doi:10.1046/j.1365-2141.1998.01121.x · Cited by 66
“The only significant differences detected between the two subgroups with and without CVD were lower Hb and higher WBC, both of which are known to signify more clinically severe SCD (Platt et al, 1994).”
BackgroundThe acute chest syndrome of sickle cell disease
Quinn, C. T., & Buchanan, G. R. (1999) · The Journal of Pediatrics · doi:10.1016/s0022-3476(99)70162-9 · Cited by 65
The initiation and progression of sickle cell nephropathy
Wesson, D. E. (2002) · Kidney International · doi:10.1046/j.1523-1755.2002.00363.x · Cited by 65
Panepinto, J. A., Magid, D. J., Rewers, M., & Lane, P. A. (2000) · The Journal of Pediatrics · doi:10.1016/s0022-3476(00)70102-8 · Cited by 65
Fitzhugh, C. D., Hsieh, M. M., Allen, D., Coles, W. A., Seamon, C., Ring, M., Zhao, X., Minniti, C. P., Rodgers, G. P., Schechter, A. N., et al. (2015) · PLOS ONE · doi:10.1371/journal.pone.0141706 · Cited by 64
“It is possible that HbF approaching 30% may be crucial to prevent organ injury and vascular damage [2].”
BackgroundResultInfluentialFetal Globin Gene Repressors as Drug Targets for Molecular Therapies To Treat the β-Globinopathies
Suzuki, M., Yamamoto, M. P., & Engel, J. D. (2014) · Molecular and Cellular Biology · doi:10.1128/mcb.00714-14 · Cited by 65
“6% was found to significantly relieve SCD mortality (20).”
BackgroundMachado, R. F., Hildesheim, M. E., Mendelsohn, L. G., Remaley, A. T., Kato, G. J., & Gladwin, M. T. (2011) · British Journal of Haematology · doi:10.1111/j.1365-2141.2011.08777.x · Cited by 63
“The CSSCD was a prospective multi-centre registry study of 3764 patients with SCD (Platt et al, 1994).”
BackgroundMethodLiver injury is associated with mortality in sickle cell disease
Feld, J. J., Kato, G. J., Koh, C., Shields, T., Hildesheim, M. E., Kleiner, D. E., Taylor, J. G., Utay, N. S., Douek, D. C., Haynes–Williams, V., et al. (2015) · Alimentary Pharmacology & Therapeutics · doi:10.1111/apt.13347 · Cited by 64
Clinical management of adult sickle-cell disease
Bartolucci, P., & Galactéros, F. (2012) · Current Opinion in Hematology · doi:10.1097/moh.0b013e328351c35f · Cited by 64
“The lengthening life expectancy of SCD patients [1,2] has increased the importance of chronic organ involvement in the functional and vital prognoses of this disease.”
BackgroundAre There Phases to the Vaso-Occlusive Painful Episode in Sickle Cell Disease?
Jacob, E., Beyer, J. E., Miaskowski, C. A., SAVEDRA, M. C., Treadwell, M. J., & Styles, L. A. (2005) · Journal of Pain and Symptom Management · doi:10.1016/j.jpainsymman.2004.07.006 · Cited by 64
Sangkatumvong, S., Khoo, M. C. K., Kato, R. M., Detterich, J. A., Bush, A., Keens, T. G., Meiselman, H. J., Wood, J. C., & Coates, T. D. (2011) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201103-0537oc · Cited by 63
Sickle Cell Disease: Management options and challenges in developing countries
Ansong, D., Osei‐Akoto, A., Ocloo, D., & Ohene‐Frempong, K. (2013) · Mediterranean Journal of Hematology and Infectious Diseases · doi:10.4084/mjhid.2013.062 · Cited by 64
“dead necrotic tissue in some parts of the body especially the bones.(4,11) Children with SCD are”
BackgroundNébor, D., Romana, M., Santiago, R., Vachiéry, N., Picot, J., Broquère, C., Chaar, V., Divialle‐Doumdo, L., Odièvre, M., Benkerrou, M., et al. (2013) · Haematologica · doi:10.3324/haematol.2012.073619 · Cited by 64
“The study may have lacked the statistical power necessary to detect those differences in the concentration of MPs shed by monocytes and granulocytes which could have been expected from the documented HC-induced decrease in the leukocyte count and in the activation level of neutrophil granulocytes.(23,33) The negative…”
BackgroundLow forced expiratory volume is associated with earlier death in sickle cell anemia
Kassim, A. A., Payne, A. B., Rodeghier, M., Macklin, E. A., Strunk, R. C., & DeBaun, M. R. (2015) · Blood · doi:10.1182/blood-2015-05-644435 · Cited by 66
Vascular Instability and Neurological Morbidity in Sickle Cell Disease: An Integrative Framework
Stotesbury, H., Kawadler, J. M., Hales, P. W., Saunders, D. E., Clark, C. A., & Kirkham, F. J. (2019) · Frontiers in Neurology · doi:10.3389/fneur.2019.00871 · Cited by 66
“In developed countries, medical advances have led to dramatically increased life expectancy for children with SCD (9).”
BackgroundChronic organ failure in adult sickle cell disease
Vichinsky, E. P. (2017) · Hematology · doi:10.1182/asheducation-2017.1.435 · Cited by 66
“At the completion of the Cooperative Study in 1988, the median age of death was 48 years for women and 42 for men.(3) Recently, the National Center for Health statistics published population-based surveillance data for all causes of death among 12 000 patients with SCD.”
BackgroundProgression of albuminuria in patients with sickle cell anemia: a multicenter, longitudinal study
Niss, O., Lane, A., Asnani, M., Yee, M. E. M., Raj, A. B., Creary, S. E., Fitzhugh, C. D., Bodas, P., Saraf, S. L., Sarnaik, S. A., et al. (2020) · Blood Advances · doi:10.1182/bloodadvances.2019001378 · Cited by 63
“Age, mean (SD) [range], y 320 21 (21) [2-64]”
Doerfler, P. A., Feng, R., Li, Y., Palmer, L. E., Porter, S. N., Bell, H. W., Crossley, M., Pruett‐Miller, S. M., Cheng, Y., & Weiss, M. J. (2021) · Nature Genetics · doi:10.1038/s41588-021-00904-0 · Cited by 67
Martín, D., Khosravi, M., Grocott, M. P. W., & Mythen, M. G. (2010) · Critical Care · doi:10.1186/cc9078 · Cited by 62
“So signifi cant is the persistence of this fetal mechanism that a low level of HbF in sickle cell patients is an independent predictor of early mortality [41] and pharmacologically increasing HbF concentration with hydroxyurea improves long-term outcome in this patient cohort [42], possibly via an NOmediated pathway […”
BackgroundInflammatory Mediators of Endothelial Injury in Sickle Cell Disease
Hoppe, C. C. (2014) · Hematology/Oncology Clinics of North America · doi:10.1016/j.hoc.2013.11.006 · Cited by 64
Gordeuk, V. R., Campbell, A., RANA, S. R., Nouraie, M. S., Niu, X., Minniti, C. P., Sable, C., Darbari, D. S., Dham, N., Onyekwere, O. C., et al. (2009) · Blood · doi:10.1182/blood-2009-04-218040 · Cited by 61
“Aspartate aminotransferase, U/L 382 40 (29-53)”
Self-citation
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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