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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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Sickle cell disease: a review for the internist
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The endothelial biology of sickle cell disease
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Fetal hemoglobin levels and morbidity in untransfused patients with β-thalassemia intermedia.
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“The majority of patients experience a poor quality of life and significantly reduced lifespan.(1,2,4) The most readily available curative option remains allogeneic hematopoietic stem cell transplantation (HSCT).”
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“Survival has increased in SCD patients in the United States and perhaps globally [4], leading to increasing identification of chronic end organ complications associated with advancing age, including pulmonary hypertension.”
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“[13] It is implicated in clinically overt stroke [14,15].”
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6 Pathophysiology of sickle cell disease
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Sickle cell disease: A distinction of two most frequent genotypes (HbSS and HbSC)
da Guarda, C. C., Yahouédéhou, S. C. M. A., Santiago, R. P., Neres, J. S. D. S., Fernandes, C. F. D. L., Aleluia, M. M., Figueiredo, C. V. B., Fiuza, L. M., Carvalho, S. P., de Oliveira, R. M., et al. (2020) · PLoS ONE · doi:10.1371/journal.pone.0228399 · Cited by 102
“In the first cluster the association of NOm and hemolytic markers reinforces the role of endothelial dysfunction [13,44], while in the second the association of leukocytes counts and CRP and AAT highlights the role of inflammation [40,41] and in the last cluster, grouping of RBC counts along with Hb, Ht and iron level…”
BackgroundMethodPatterns of mortality in sickle cell disease in adults in France and England
Perronne, V., Roberts-Harewood, M., Bachir, D., Roudot‐Thoraval, F., Delord, J.-M., Thuret, I., Schaeffer, A., Davies, S. C., Galactéros, F., & Godeau, B. (2002) · The Hematology Journal · doi:10.1038/sj.thj.6200147 · Cited by 102
Longitudinal Decline in Lung Volume in a Population of Children with Sickle Cell Disease
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“It is a multisystem chronic disease that results in a decreased life span, with asignificantincreasein mortalitybeginninginthethirddecadeof life (1, 2 ). Mortality attributable to pulmonary disease is significant, with estimates ranging from 21 to 85% (2‐4).”
BackgroundMethodResultInfluentialThe Perfusion Paradox and Vascular Instability in Sickle Cell Disease
NATH, K. A., KATUSIC, Z. S., & GLADWIN, M. T. (2004) · Microcirculation · doi:10.1080/10739680490278592 · Cited by 102
“Renal disease, as reflected by proteinuria, occurs in some 30% of patients while progressive decline in renal function leading to endstage renal disease occurs in up to 18% of patients (94,100), the latter substantially increasing morbidity and mortality observed in these patients (1,93,94).”
BackgroundGenetics of fetal hemoglobin in Tanzanian and British patients with sickle cell anemia
Makani, J., Menzel, S., Nkya, S. W., Cox, S. E., Drašar, E., Soka, D., Komba, A., Mgaya, J., Rooks, H., Vasavda, N., et al. (2010) · Blood · doi:10.1182/blood-2010-08-302703 · Cited by 103
“To uncover new loci and variants controlling HbF in populations where SCA is endemic, genetic studies focused on individual African populations may be more informative, and patient resources across Africa, such as the Muhimbili Sickle Cell Collaborative Program, can make important contributions toward this goal.”
InfluentialPulmonary Complications of Sickle Cell Anemia
Minter, K. R., & Gladwin, M. T. (2001) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/ajrccm.164.11.2104101 · Cited by 102
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“for men and women with sickle cell anemia was 42 and 48 years, respectively [4].”
BackgroundSelf-citationPrevelance and clinical correlates of glomerulopathy in children with sickle cell disease
Wigfall, D. R., Ware, R. E., Burchinal, M., Kinney, T. R., & Foreman, J. W. (2000) · The Journal of Pediatrics · doi:10.1016/s0022-3476(00)48828-1 · Cited by 101
Johnson, M. C., Kirkham, F. J., Redline, S. S., ROSEN, C. L., Yan, Y., Roberts, I. A. G., Gruenwald, J., Marek, J., & DeBaun, M. R. (2010) · Blood · doi:10.1182/blood-2009-06-227447 · Cited by 99
“Sleep variables included (1) average oxygen saturation asleep; (2) percentage of sleep time with oxygen saturation less than 95%; (3) obstructive apnea hypopnea index (OAHI), that is, the count of all obstructive apneas regardless of associated desaturation plus the count of hypopneas associated with at least a 3% oxy…”
MethodSix Months of Hydroxyurea Reduces Albuminuria in Patients with Sickle Cell Disease
Bartolucci, P., Habibi, A., Stehlé, T., Liberto, G. D., Rakotoson, M. G., Gellen‐Dautremer, J., Loric, S., Moutereau, S., Sahali, D., Wagner‐Ballon, O., et al. (2015) · Journal of the American Society of Nephrology · doi:10.1681/asn.2014111126 · Cited by 100
Jones, R. J., & DeBaun, M. R. (2021) · Blood · doi:10.1182/blood.2021011488 · Cited by 102
Identification of non-coding silencer elements and their regulation of gene expression
Pang, B., van Weerd, J. H., Hamoen, F. L., & Snyder, M. P. (2022) · Nature Reviews Molecular Cell Biology · doi:10.1038/s41580-022-00549-9 · Cited by 103
Pulmonary function abnormalities in children with sickle cell disease.
Sylvester, K. P., Patey, R. A., Milligan, P. J., Dick, M. C., Rafferty, G. F., Rees, D. C., Thein, S. L., & Greenough, A. (2004) · PubMed Central · Cited by 100
Mortality in sickle cell patients on hydroxyurea therapy
Bakanay, Ş. M., Dainer, E., Clair, B., Adekile, A. D., Daitch, L., Wells, L., Holley, L., Smith, D. A., & Kutlar, A. (2004) · Blood · doi:10.1182/blood-2004-01-0322 · Cited by 100
“The mortality and morbidity in sickle cell disease (SCD) have been considerably reduced since the introduction of hydroxyurea in the 1990’s ((1-3)).”
BackgroundOkpala, I. E., Daniel, Y., Haynes, R., Odoemene, D., & Goldman, J. M. (2002) · European Journal Of Haematology · doi:10.1034/j.1600-0609.2002.02775.x · Cited by 100
“with high disease-related mortality (11).”
BackgroundIron, Inflammation, and Early Death in Adults With Sickle Cell Disease
van Beers, E. J., Yang, Y., Raghavachari, N., Tian, X., Allen, D. T., Nichols, J. S., Mendelsohn, L. G., Nekhai, S. A., Gordeuk, V. R., Taylor, J. G., et al. (2014) · Circulation Research · doi:10.1161/circresaha.116.304577 · Cited by 99
“Although no direct association with CRP and mortality has been described, the long-standing recognition that an elevated leukocyte count predicts early death in SCD suggests a possible relationship of inflammation to mortality.(8) Three mechanisms cause a high exposure of SCD peripheral blood mononuclear cells (PBMCs)…”
BackgroundSickle cell disease: Clinical presentation and management of a global health challenge
Houwing, M. E., de Pagter, P. J., van Beers, E. J., Biemond, B. J., Rettenbacher, E., Rijneveld, A. W., Schols, E. M., Philipsen, J. N. J., Tamminga, R. Y. J., van Draat, K. F., et al. (2019) · Blood Reviews · doi:10.1016/j.blre.2019.05.004 · Cited by 106
Enalapril and hydroxyurea therapy for children with sickle nephropathy
Fitzhugh, C. D., Wigfall, D. R., & Ware, R. E. (2005) · Pediatric Blood & Cancer · doi:10.1002/pbc.20296 · Cited by 99
“Proteinuria in SCA frequently progresses to nephrotic syndrome [3] and ultimately to renal failure [1,6], which is a common cause of mortality among adults with SCA [7,8].”
BackgroundRedox-dependent impairment of vascular function in sickle cell disease
Aslan, M., & Freeman, B. Α. (2007) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2007.08.014 · Cited by 97
“The participation of leukocytes in the pathophysiology of sickle cell disease is supported by large epidemiologic studies that show high baseline leukocyte counts as a risk factor for disease severity (42), acute chest syndrome (43) and silent cerebral infarcts (44).”
BackgroundPulmonary Hypertension in Children and Adolescents with Sickle Cell Disease
Onyekwere, O. C., Campbell, A., Teshome, M., Onyeagoro, S., Sylvan, C., Akintilo, A., Hutchinson, S. K., Ensing, G. J., Gaskin, P. R. A., Kato, G. J., et al. (2007) · Pediatric Cardiology · doi:10.1007/s00246-007-9018-x · Cited by 99
Self-citationTargeted Therapeutic Strategies for Fetal Hemoglobin Induction
Sankaran, V. G. (2011) · Hematology · doi:10.1182/asheducation-2011.1.459 · Cited by 102
Embryonic and fetal β‐globin gene repression by the orphan nuclear receptors, TR2 and TR4
Tanabe, O., McPhee, D., Kobayashi, S., Shen, Y., Brandt, W., Jiang, X., Campbell, A. D., Chen, Y., Chang, C. S., Yamamoto, M., et al. (2007) · The EMBO Journal · doi:10.1038/sj.emboj.7601676 · Cited by 99
“A previous clinical study showed that sickle-cell patients with higher (48.6%) HbF levels experience significantly reduced mortality ( Platt et al, 1994 ).”
BackgroundInfluentialPule, G. D., Mowla, S., Novitzky, N., Wiysonge, C. S., & Wonkam, A. (2015) · Expert Review of Hematology · doi:10.1586/17474086.2015.1078235 · Cited by 103
“Indeed, despite the fact that there are several key phenotypes of SCD (anemia, stroke, infections), fetal hemoglobin (HbF) has emerged as a central disease modifier [10], that is amenable to therapeutic manipulation [11].”
BackgroundSickle cell nephropathy – a practical approach
Sharpe, C. C., & Thein, S. L. (2011) · British Journal of Haematology · doi:10.1111/j.1365-2141.2011.08853.x · Cited by 97
“…with other complications, renal dysfunction is relatively more common and more severe in those with HbSS and HbSb0 thalassaemia genotypes (sickle cell anaemia, SCA) than in those with HbSC or HbSb+ thalassaemia, which are generally milder forms of SCD (Platt et al, 1994; Guasch et al, 2006).”
BackgroundAspectos epidemiológicos e sociais da doença falciforme
Felix, A. A., Moraes‐Souza, H., & Ribeiro, S. B. M. H. A. (2010) · Revista Brasileira de Hematologia e Hemoterapia · doi:10.1590/s1516-84842010005000072 · Cited by 97
Krishnamurti, L., Neuberg, D. S., Sullivan, K. M., Kamani, N. R., Abraham, A., Campigotto, F., Zhang, W., Dahdoul, T., Castro, L. D., Parikh, S. H., et al. (2019) · American Journal of Hematology · doi:10.1002/ajh.25401 · Cited by 97
“Day 180 cumulative incidence grades II-IV acute GVHD [95% CI] 18 [5-37] 18 [4-39] 20 [1-62]”
A molecular glue degrader of the WIZ transcription factor for fetal hemoglobin induction
Ting, P. Y., Borikar, S., Kerrigan, J. R., Thomsen, N. M., Aghania, E., Hinman, A. E., Reyes, A., Pizzato, N., Fodor, B. D., Wu, F., et al. (2024) · Science · doi:10.1126/science.adk6129 · Cited by 97
Fludarabine-based conditioning for allogeneic transplantation in adults with sickle cell disease
van Besien, K., Bartholomew, A. M., Stock, W., Peace, D. J., Devine, S. M., Sher, D., Sosman, J. A., Chen, Y.-H., Koshy, M., & Hoffman, R. (2000) · Bone Marrow Transplantation · doi:10.1038/sj.bmt.1702518 · Cited by 97
Asthma and acute chest in sickle‐cell disease
Boyd, J. H., Moinuddin, A., Strunk, R. C., & DeBaun, M. R. (2004) · Pediatric Pulmonology · doi:10.1002/ppul.20066 · Cited by 97
“Further, ACS is the leading cause of premature death in this population and affects approximately 50% of patients with sickle-cell anemia at least once in their lifetime.(1,2) The etiology of ACS is multifactorial, and the pathogenesis is not completely understood.”
BackgroundInfluentialAlbuminuria and Renal Function in Homozygous Sickle Cell Disease
Thompson, J. (2007) · Archives of Internal Medicine · doi:10.1001/archinte.167.7.701 · Cited by 97
Quality-of-Care Indicators for Children With Sickle Cell Disease
Wang, C.-H. J., Kavanagh, P. L., Little, A. A., Holliman, J. B., & Sprinz, P. G. (2011) · PEDIATRICS · doi:10.1542/peds.2010-1791 · Cited by 97
ZNF410 Uniquely Activates the NuRD Component CHD4 to Silence Fetal Hemoglobin Expression
Lan, X., Ren, R., Feng, R., Ly, L. C., Lan, Y., Zhang, Z., Aboreden, N. G., Qin, K., Horton, J. R., Grevet, J. D., et al. (2020) · Molecular Cell · doi:10.1016/j.molcel.2020.11.006 · Cited by 95
Splenic Complications of Sickle Cell Anemia and the Role of Splenectomy
Al-Salem, A. H. (2010) · ISRN Hematology · doi:10.5402/2011/864257 · Cited by 97
Induction of Fetal Hemoglobin in the Treatment of Sickle Cell Disease
Fathallah, H., & Atweh, G. F. (2006) · Hematology · doi:10.1182/asheducation-2006.1.58 · Cited by 95
“Moreover, the Cooperative Study of Sickle Cell Disease identified HbF as a major prognostic factor for several clinical complications including painful events,(2) acute chest syndrome(3) and death.(4) These clinical and epidemiological observations provided important clues about the beneficial role of HbF in ameliorat…”
BackgroundSecretory phospholipase A2 predicts impending acute chest syndrome in sickle cell disease
Styles, L. A., Aarsman, A. J., Vichinsky, E. P., & Kuypers, F. A. (2000) · Blood · doi:10.1182/blood.v96.9.3276 · Cited by 105
Waugh, W. H., Daeschner, C. W., Files, B., McConnell, M. E., & Strandjord, S. E. (2001) · PubMed Central · Cited by 95
Microvascular abnormalities in sickle cell disease: a computer-assisted intravital microscopy study
Cheung, A., Chen, P. C. Y., Larkin, E. C., Duong, P. L., Ramanujam, S., Tablin, F., & Wun, T. (2002) · Blood · doi:10.1182/blood.v99.11.3999 · Cited by 95
The Excess Burden of Stroke in Hospitalized Adults with Sickle Cell Disease
Strouse, J. J., Jordan, L. C., Lanzkron, S., & Casella, J. (2009) · American journal of hematology/oncology · doi:10.1002/ajh.21476 · Cited by 102
“Our estimates of survival were similar to those obtained for sickle cell anemia from the CSSCD [34] and a large Californian cohort study [13], but about 10 years less than a Jamaican cohort study that adjusted for excess mortality before registration in the cohort [35].”
InfluentialSystemic Exposure to Morphine and the Risk of Acute Chest Syndrome in Sickle Cell Disease
Kopecky, E. A., Jacobson, S., Joshi, P., & Koren, G. (2004) · Clinical Pharmacology & Therapeutics · doi:10.1016/j.clpt.2003.10.007 · Cited by 95
Hemker, B. G., Brousseau, D. C., Yan, K., Hoffmann, R. G., & Panepinto, J. A. (2011) · American Journal of Hematology · doi:10.1002/ajh.22106 · Cited by 95
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Malignancy in patients with sickle cell disease
Schultz, W. H., & Ware, R. E. (2003) · American Journal of Hematology · doi:10.1002/ajh.10427 · Cited by 96
“The development of malignancy in persons with sickle cell disease (SCD) has been documented by numerous individual cases [1–10] and several small series [11–13] over the past 50 years.”
Cytokines in Sickle Cell Disease
Pathare, A. V., Alkindi, S. S. A., Daar, S., & Dennison, D. (2003) · Hematology · doi:10.1080/10245330310001604719 · Cited by 95
“Firstly, a high PMN count is a characteristic feature of SCD patients, and polymorphonuclear leukocytosis is related to an increased risk of early death, ACSs and stroke [92,93].”
BackgroundAdhesion of sickle neutrophils and erythrocytes to fibronectin
Kasschau, M., Barabino, G., Bridges, K., & Golan, D. (1996) · Blood · doi:10.1182/blood.v87.2.771.bloodjournal872771 · Cited by 94
Self-Efficacy as a Predictor of Adult Adjustment to Sickle Cell Disease: One-Year Outcomes
Edwards, R. R., Telfair, J., Cecil, H., & Lenoci, J. M. (2001) · Psychosomatic Medicine · doi:10.1097/00006842-200109000-00020 · Cited by 94
“Because most children with SCD now survive far into adulthood (13, 14), researchers need to examine psychosocial factors that affect adjustment to SCD among adults as well as children.”
BackgroundSickle Cell Disease in Children
Meier, E. R., & Miller, J. L. (2012) · Drugs · doi:10.2165/11632890-000000000-00000 · Cited by 97
“First approved for treatment of iron overload in bthalassaemia patients, deferasirox 20mg/kg/day showed non-inferiority in maintaining serum ferritin and reducing liver iron content (LIC) compared with deferoxamine when used in patients with SCD.Deferasirox doses of 30mg/kg/day reduced both serum ferritin and LIC.”
BackgroundInfluential
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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