Acute lymphoblastic leukemia: an assessment of international incidence, survival, and disease burden
Acute lymphoblastic leukemia (ALL) is a rare hematological malignancy. With the recent introduction of a classification system for hematopoietic and lymphoid neoplasms, more comprehensive assessment of ALL epidemiology is now possible. In this study, we describe recent international incidence of ALL and project the annual number of diagnoses to 2025. We also estimate relative survival and average potential years of life lost (AYLL) to assess the societal burden of ALL.
Age-specific incidence data for ALL from select cancer registries in different geographies were obtained from the International Agency for Research on Cancer’s Cancer Incidence in Five Continents Database. Country-specific age-standardized rates were calculated to allow for direct comparisons between countries. ALL-specific mortality and relative survival data were only available from the United States (US) National Cancer Institute’s Surveillance, Epidemiology, and End Results program; mortality rates were estimated for other countries.
The age-standardized incidence rate of ALL during 2003–2007 ranged from 1.08 to 2.12 per 100,000 person-years in selected countries. Incidence was generally higher in the Americas and Oceania and lower in Asia and Eastern Europe. In most countries, the incidence rate of ALL in children was approximately four times that in adults. Survival was particularly poor among adults. In selected countries, the estimated AYLL ranged from 30 to 48 years for all ages and from 23 to 39 years for adults.
Although a rare disease, ALL presents a significant public health burden given poor survival outcomes among adults, AYLL, and its importance as the most common pediatric cancer.
KeywordsAcute lymphoblastic leukemia International Incidence Projections Survival Potential years of life lost
Compliance with ethical standards
The manuscript does not contain clinical studies, patient data, or research involving human participants or animals.
Conflict of interest
A.J.K., V.M.C., and M.A.K. are employees of Amgen Inc. W.M.S. is an employee of Amgen Ltd.
- 3.Hunger SP, Lu X, Devidas M, Camitta BM, Gaynon PS, Winick NJ et al (2012) Improved survival for children and adolescents with acute lymphoblastic leukemia between 1990 and 2005: a report from the children’s oncology group. J Clin Oncol 30:1663–1669. doi: 10.1200/jco.2011.37.8018 PubMedCentralCrossRefPubMedGoogle Scholar
- 5.Rowe JM, Buck G, Burnett AK, Chopra R, Wiernik PH, Richards SM et al (2005) Induction therapy for adults with acute lymphoblastic leukemia: results of more than 1500 patients from the international ALL trial: MRC UKALL XII/ECOG E2993. Blood 106:3760–3767. doi: 10.1182/blood-2005-04-1623 CrossRefPubMedGoogle Scholar
- 7.Sive JI, Buck G, Fielding A, Lazarus HM, Litzow MR, Luger S et al (2012) Outcomes in older adults with acute lymphoblastic leukaemia (ALL): results from the international MRC UKALL XII/ECOG2993 trial. Br J Haematol 157:463–471. doi: 10.1111/j.1365-2141.2012.09095.x PubMedCentralCrossRefPubMedGoogle Scholar
- 8.Thomas DA, O’Brien S, Faderl S, Garcia-Manero G, Ferrajoli A, Wierda W et al (2010) Chemoimmunotherapy with a modified hyper-CVAD and rituximab regimen improves outcome in de novo Philadelphia chromosome-negative precursor B-lineage acute lymphoblastic leukemia. J Clin Oncol 28:3880–3889. doi: 10.1200/jco.2009.26.9456 PubMedCentralCrossRefPubMedGoogle Scholar
- 9.Oriol A, Vives S, Hernandez-Rivas JM, Tormo M, Heras I, Rivas C et al (2010) Outcome after relapse of acute lymphoblastic leukemia in adult patients included in four consecutive risk-adapted trials by the PETHEMA Study Group. Haematologica 95:589–596. doi: 10.3324/haematol.2009.014274 PubMedCentralCrossRefPubMedGoogle Scholar
- 11.Gökbuget N, Stanze D, Beck J, Diedrich H, Horst HA, Huttmann A et al (2012) Outcome of relapsed adult lymphoblastic leukemia depends on response to salvage chemotherapy, prognostic factors, and performance of stem cell transplantation. Blood 120:2032–2041. doi: 10.1182/blood-2011-12-399287 CrossRefPubMedGoogle Scholar
- 13.American Cancer Society (2011) Global cancer facts and figures, 2nd edn. American Cancer Society, AtlantaGoogle Scholar
- 22.Marcos-Gragera R, Allemani C, Tereanu C, De Angelis R, Capocaccia R, Maynadie M et al (2011) Survival of European patients diagnosed with lymphoid neoplasms in 2000–2002: results of the HAEMACARE project. Haematologica 96:720–728. doi: 10.3324/haematol.2010.034264 PubMedCentralCrossRefPubMedGoogle Scholar
- 27.Coebergh JW, Reedijk AM, de Vries E, Martos C, Jakab Z, Steliarova-Foucher E et al (2006) Leukaemia incidence and survival in children and adolescents in Europe during 1978–1997. Report from the Automated Childhood Cancer Information System project. Eur J Cancer 42:2019–2036. doi: 10.1016/j.ejca.2006.06.005 CrossRefPubMedGoogle Scholar
- 28.Fajardo-Gutierrez A, Juarez-Ocana S, Gonzalez-Miranda G, Palma-Padilla V, Carreon-Cruz R, Ortega-Alvarez MC et al (2007) Incidence of cancer in children residing in ten jurisdictions of the Mexican Republic: importance of the Cancer registry (a population-based study). BMC Cancer 7:68. doi: 10.1186/1471-2407-7-68 PubMedCentralCrossRefPubMedGoogle Scholar
- 30.Forman D, Bray F, Brewster DH, Gombe Mbalawa C, Kholer B, Piñeros M et al (2013) Cancer Incidence in Five Continents Volume X (IARC Scientific Publications No. 164). Lyon, IARCGoogle Scholar
- 31.Ahmad OB, Boschi-Pinto C, López AD, Murray CJLLR, Inoue M (2001) Age standardization of rates: a new WHO standard. World Health Organization, GenevaGoogle Scholar
- 32.United Nations, Department of Economic and Social Affairs, Population Division (2013) World Population Prospects: The 2012 Revision. http://esa.un.org/wpp/unpp/panel_indicators.htm. Accessed Feb 21, 2014
- 33.Chiaretti S, Vitale A, Cazzaniga G, Orlando SM, Silvestri D, Fazi P et al (2013) Clinico-biological features of 5202 patients with acute lymphoblastic leukemia enrolled in the Italian AIEOP and GIMEMA protocols and stratified in age cohorts. Haematologica 98:1702–1710. doi: 10.3324/haematol.2012.080432 PubMedCentralCrossRefPubMedGoogle Scholar
- 36.Chen B, Wang YY, Shen Y, Zhang WN, He HY, Zhu YM et al (2012) Newly diagnosed acute lymphoblastic leukemia in China (I): abnormal genetic patterns in 1346 childhood and adult cases and their comparison with the reports from Western countries. Leukemia 26:1608–1616. doi: 10.1038/leu.2012.26 CrossRefPubMedGoogle Scholar
- 38.Moorman AV, Harrison CJ, Buck GA, Richards SM, Secker-Walker LM, Martineau M et al (2007) Karyotype is an independent prognostic factor in adult acute lymphoblastic leukemia (ALL): analysis of cytogenetic data from patients treated on the Medical Research Council (MRC) UKALLXII/Eastern Cooperative Oncology Group (ECOG) 2993 trial. Blood 109:3189–3197. doi: 10.1182/blood-2006-10-051912 CrossRefPubMedGoogle Scholar
- 40.Kantarjian H, Thomas D, O’Brien S, Cortes J, Giles F, Jeha S et al (2004) Long-term follow-up results of hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone (Hyper-CVAD), a dose-intensive regimen, in adult acute lymphocytic leukemia. Cancer 101:2788–2801. doi: 10.1002/cncr.20668 CrossRefPubMedGoogle Scholar
- 41.Surveillance, Epidemiology, and End Results (SEER) Program (www.seer.cancer.gov) SEER*Stat Database: Incidence—SEER 18 Regs Research Data + Hurrican Katrina Impacted louisiana Cases, Nov 2013 Sub (1973–2011 varying)—Linked to County Attributes—Total U.S., 1969–2012 Counties, National Cancer Instisute, DCCPS, Surveillance Research Program, Surveillance Systems Branch, released April 2014 (updated 5/7/2017), based on the November 2013 submission
- 42.Surveillance, Epidemiology, and End Results (SEER) Program (www.seer.cancer.gov) SEER*Stat Database: Mortality—All COD, Aggregated With State, Total U.S. (1969–2010) <Katrina/Rita Population Adjustment>, National Cancer Instisute, DCCPS, Surveillance Research Program, Surveillance Systems Branch, released April 2013. Underlying mortality data provided by NCHS (www.cdc.gov/nchs)
- 43.World Health Organization (2013) Global Health Observatory Data Repository. http://apps.who.int/ghodata/. Accessed May 19, 2014
- 45.Office for National Statistics (2015) Causes of Death. http://www.ons.gov.uk/ons/taxonomy/index.html?nscl=Causes+of+Death#tab-data-tables. Accessed Jan 8, 2015
- 46.NHS National Services Scotland, Information Services Division (2015) Data tables: cancer mortality by year. http://www.isdscotland.org/Health-Topics/Cancer/Publications/data-tables.asp?id=1314#1314. Accessed Jan 8, 2015
- 47.Instituto Nacional de Estadística (2015) Health: Deaths statistic according to cause of death. http://www.ine.es/jaxi/menu.do?type=pcaxis&path=%2Ft15%2Fp417&file=inebase&L=1. Accessed Jan 8, 2015
- 48.Australian Institute of Health and Welfare (AIHW) (2014) Australian Cancer Incidence and Mortality (ACIM) books: acute lymphoblastic leukemia. AIHW, Canberra. http://www.aihw.gov.au/acim-books. Accessed Jan 8, 2015
- 49.Ehngholm G, Ferlay J, Christensen N, Kejs AMT, Johannesen TB, Khan S, et al (2014) NORDCAN: Cancer incidence, mortality, prevalence and survival in the Nordic countries, version 7.0. Association of the Nordic Cancer Registries. Danish Cancer Society. http://www.ancr.nu. Accessed Jan 8, 2015
- 57.Sant M, Minicozzi P, Mounier M, Anderson LA, Brenner H, Holleczek B et al (2014) Survival for haematological malignancies in Europe between 1997 and 2008 by region and age: results of EUROCARE-5, a population-based study. Lancet Oncol 15:931–942. doi: 10.1016/s1470-2045(14)70282-7 CrossRefPubMedGoogle Scholar
- 58.Horibe K, Saito AM, Takimoto T, Tsuchida M, Manabe A, Shima M et al (2013) Incidence and survival rates of hematological malignancies in Japanese children and adolescents (2006–2010): based on registry data from the Japanese Society of Pediatric Hematology. Int J Hematol 98:74–88. doi: 10.1007/s12185-013-1364-2 CrossRefPubMedGoogle Scholar