{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,12]],"date-time":"2026-11-12T02:47:28Z","timestamp":1794451648230,"version":"4.9.1"},"reference-count":57,"publisher":"American Society of Hematology","issue":"1","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2024,12,6]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>India, the most populous nation in the world, also has a high frequency of the sickle hemoglobin (HbS) allele globally. The Arab Indian HbS haplotype in India is characterized by a relatively high percentage of fetal Hb, with widely varying frequencies of \u03b1-thalassemia. Hence, sickle cell disease (SCD) in India was perceived to be mild. Advances in the past decade in screening and SCD management have revealed that the severity of SCD in India is comparable to many other parts of the world. Clinical features in India include vaso-occlusive crisis, acute chest syndrome, avascular necrosis, renal involvement, stroke, etc, at a relatively young age. Once a fatal disease of childhood, the majority of patients born with SCD are expected to survive into adulthood, largely because of improvements in comprehensive care programs including newborn screening, penicillin prophylaxis, transcranial Doppler, and hydroxyurea therapy. Several centers are performing hematopoietic stem cell transplants successfully for SCD. To address the urgent need to control and manage SCD in India's population, the Government of India launched the National Sickle Cell Anaemia Elimination Mission, with significant funding for large-scale measures to screen, treat, counsel, educate, and develop technologies and novel therapies and gene therapies.<\/jats:p>","DOI":"10.1182\/hematology.2024000678","type":"journal-article","created":{"date-parts":[[2024,12,6]],"date-time":"2024-12-06T15:01:47Z","timestamp":1733497307000},"page":"1-9","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":26,"title":["Sickle cell disease in India: the journey and hope for the future"],"prefix":"10.1182","volume":"2024","author":[{"given":"Kalpna","family":"Gupta","sequence":"first","affiliation":[{"name":"1 Hematology\/Oncology Division, Department of Medicine, University of California, Irvine, CA"},{"name":"2 Division of Hematology, Oncology and Transplantation, Department of Medicine, University of Minnesota, Minneapolis, MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lakshmanan","family":"Krishnamurti","sequence":"additional","affiliation":[{"name":"3 Pediatric Hematology and Oncology, Yale School of Medicine, New Haven, CT"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dipty","family":"Jain","sequence":"additional","affiliation":[{"name":"4 Department of Paediatrics, Government Medical College, Nagpur, Maharashtra, India"},{"name":"5 Datta Meghe Medical College, Nagpur, Maharashtra, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"234","published-online":{"date-parts":[[2024,12,6]]},"reference":[{"issue":"8","key":"2024121115353654000_hem2024000678C1","doi-asserted-by":"crossref","first-page":"e585","DOI":"10.1016\/S2352-3026(23)00118-7","article-title":"Global, regional, and national prevalence and mortality burden of sickle cell disease, 2000-2021: a systematic analysis from the Global Burden of Disease Study 2021","volume":"10","author":"GBDSCD Collaborators","year":"2023","journal-title":"Lancet Haematol"},{"issue":"1","key":"2024121115353654000_hem2024000678C2","doi-asserted-by":"crossref","first-page":"17685","DOI":"10.1038\/s41598-018-36077-w","article-title":"The spatial epidemiology of sickle-cell anaemia in India","volume":"8","author":"Hockham","year":"2018","journal-title":"Sci Rep"},{"issue":"9861","key":"2024121115353654000_hem2024000678C3","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1016\/S0140-6736(12)61229-X","article-title":"Global epidemiology of sickle haemoglobin in neonates: a contemporary geostatistical model-based map and population estimates","volume":"381","author":"Piel","year":"2013","journal-title":"Lancet"},{"issue":"6","key":"2024121115353654000_hem2024000678C4","doi-asserted-by":"crossref","first-page":"491","DOI":"10.4103\/ijmr.ijmr_1045_23","article-title":"A new era dawns on sickle cell disease in India","volume":"157","author":"Kishore","year":"2023","journal-title":"Indian J Med Res"},{"issue":"3","key":"2024121115353654000_hem2024000678C5","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1097\/MOH.0000000000000422","article-title":"Clinical manifestations of sickle cell disease in India: misconceptions and reality","volume":"25","author":"Jain","year":"2018","journal-title":"Curr Opin Hematol"},{"issue":"8","key":"2024121115353654000_hem2024000678C6","doi-asserted-by":"crossref","first-page":"795","DOI":"10.1002\/ajh.23232","article-title":"Genetic modifiers of sickle cell disease","volume":"87","author":"Steinberg","year":"2012","journal-title":"Am J Hematol"},{"issue":"16","key":"2024121115353654000_hem2024000678C7","doi-asserted-by":"crossref","first-page":"1561","DOI":"10.1056\/NEJMra1510865","article-title":"Sickle cell disease","volume":"376","author":"Piel","year":"2017","journal-title":"N Engl J Med"},{"issue":"1","key":"2024121115353654000_hem2024000678C8","doi-asserted-by":"crossref","first-page":"5","DOI":"10.4103\/jfmpc.jfmpc_398_23","article-title":"How healthy is the health budget of Amrit Kaal: 2023-24?","volume":"13","author":"Majhi","year":"2024","journal-title":"J Family Med Prim Care"},{"key":"2024121115353654000_hem2024000678C9","unstructured":"Ministry of Health and Family Welfare, Government of India. National Sickle Cell Anaemia Elimination Mission 2023. Accessed 13July2024. https:\/\/sickle.nhm.gov.in\/."},{"issue":"4755","key":"2024121115353654000_hem2024000678C10","doi-asserted-by":"crossref","first-page":"404","DOI":"10.1136\/bmj.1.4755.404","article-title":"Sickle-cell trait in southern India","volume":"1","author":"Lehmann","year":"1952","journal-title":"Br Med J"},{"issue":"9","key":"2024121115353654000_hem2024000678C11","first-page":"387","article-title":"The occurrence of sickle cell anaemia among a group of tea garden labourers in Upper Assam","volume":"87","author":"Dunlop","year":"1952","journal-title":"Ind Med Gaz"},{"issue":"7015","key":"2024121115353654000_hem2024000678C12","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1016\/S0140-6736(58)91035-3","article-title":"Sickle-cell trait in Central India","volume":"1","author":"Shukla","year":"1958","journal-title":"Lancet"},{"issue":"11","key":"2024121115353654000_hem2024000678C13","doi-asserted-by":"crossref","first-page":"911","DOI":"10.1007\/s13312-012-0217-z","article-title":"Sickle cell anemia from central India: a retrospective analysis","volume":"49","author":"Jain","year":"2012","journal-title":"Indian Pediatr"},{"issue":"3","key":"2024121115353654000_hem2024000678C14","first-page":"317","article-title":"Morbidity pattern in hospitalized under five children with sickle cell disease","volume":"138","author":"Jain","year":"2013","journal-title":"Indian J Med Res"},{"issue":"10","key":"2024121115353654000_hem2024000678C15","doi-asserted-by":"crossref","first-page":"1814","DOI":"10.1002\/pbc.26085","article-title":"Clinical events in a large prospective cohort of children with sickle cell disease in Nagpur, India: evidence against a milder clinical phenotype in India","volume":"63","author":"Jain","year":"2016","journal-title":"Pediatr Blood Cancer"},{"issue":"4","key":"2024121115353654000_hem2024000678C16","doi-asserted-by":"crossref","first-page":"326","DOI":"10.4103\/ijmr.IJMR_1946_18","article-title":"Homozygous sickle cell disease in Central India and Jamaica: a comparison of newborn cohorts","volume":"151","author":"Jain","year":"2020","journal-title":"Indian J Med Res"},{"issue":"4","key":"2024121115353654000_hem2024000678C17","first-page":"479","article-title":"Haplotypes in tribal Indians bearing the sickle gene: evidence for the unicentric origin of the beta S mutation and the unicentric origin of the tribal populations of India","volume":"61","author":"Labie","year":"1989","journal-title":"Hum Biol"},{"key":"2024121115353654000_hem2024000678C18","first-page":"1368","article-title":"Clinical profile of sickle cell trait","volume":"50","author":"Kar","year":"2002","journal-title":"J Assoc Physicians India"},{"issue":"2","key":"2024121115353654000_hem2024000678C19","first-page":"255","article-title":"Priapism is rare in sickle cell disease in India","volume":"48","author":"Dash","year":"2000","journal-title":"J Assoc Physicians India"},{"issue":"9","key":"2024121115353654000_hem2024000678C20","first-page":"890","article-title":"Splenectomy in sickle cell disease","volume":"47","author":"Kar","year":"1999","journal-title":"J Assoc Physicians India"},{"issue":"1","key":"2024121115353654000_hem2024000678C21","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1007\/BF02795780","article-title":"Clinical profile of sickle cell disease in Orissa","volume":"64","author":"Kar","year":"1997","journal-title":"Indian J Pediatr"},{"issue":"7","key":"2024121115353654000_hem2024000678C22","first-page":"418","article-title":"Serum immunoglobulins in sickle cell disease","volume":"41","author":"Mohapatra","year":"1993","journal-title":"J Assoc Physicians India"},{"issue":"12","key":"2024121115353654000_hem2024000678C23","first-page":"954","article-title":"Sickle cell disease in India","volume":"39","author":"Kar","year":"1991","journal-title":"J Assoc Physicians India"},{"issue":"8","key":"2024121115353654000_hem2024000678C24","first-page":"555","article-title":"Sickle haemoglobin, G-6PD deficiency and malaria in western Orissa","volume":"38","author":"Kar","year":"1990","journal-title":"J Assoc Physicians India"},{"key":"2024121115353654000_hem2024000678C25","first-page":"427","article-title":"Raised Hb F levels in sickle cell disease are caused by a determinant linked to the beta globin gene cluster","volume":"251","author":"Kulozik","year":"1987","journal-title":"Prog Clin Biol Res"},{"issue":"5","key":"2024121115353654000_hem2024000678C26","doi-asserted-by":"crossref","first-page":"e322","DOI":"10.1016\/S2352-3026(24)00109-1","article-title":"Sickle cell disease in India: current status and progress","volume":"11","author":"Jain","year":"2024","journal-title":"Lancet Haematol"},{"issue":"6","key":"2024121115353654000_hem2024000678C27","doi-asserted-by":"crossref","first-page":"505","DOI":"10.4103\/ijmr.IJMR_2064_20","article-title":"Sickle cell disease: progress made and challenges ahead","volume":"151","author":"Odame","year":"2020","journal-title":"Indian J Med Res"},{"issue":"1","key":"2024121115353654000_hem2024000678C28","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1007\/s12288-018-0994-1","article-title":"Red cell indices and hemoglobin profile of newborn babies with both the sickle gene and alpha thalassaemia in Central India","volume":"35","author":"Upadhye","year":"2019","journal-title":"Indian J Hematol Blood Transfus"},{"issue":"1","key":"2024121115353654000_hem2024000678C29","doi-asserted-by":"crossref","first-page":"e0147081","DOI":"10.1371\/journal.pone.0147081","article-title":"Neonatal screening and the clinical outcome in children with sickle cell disease in Central India","volume":"11","author":"Upadhye","year":"2016","journal-title":"PLoS One"},{"issue":"7","key":"2024121115353654000_hem2024000678C30","doi-asserted-by":"crossref","first-page":"1201","DOI":"10.1007\/s00277-016-2675-1","article-title":"Influence of single nucleotide polymorphisms in the BCL11A and HBS1L-MYB gene on the HbF levels and clinical severity of sickle cell anaemia patients","volume":"95","author":"Upadhye","year":"2016","journal-title":"Ann Hematol"},{"issue":"10","key":"2024121115353654000_hem2024000678C31","doi-asserted-by":"crossref","first-page":"1071","DOI":"10.1007\/s12098-016-2081-7","article-title":"Sickle cell disease in Central India: a potentially severe syndrome","volume":"83","author":"Jain","year":"2016","journal-title":"Indian J Pediatr"},{"issue":"2","key":"2024121115353654000_hem2024000678C32","doi-asserted-by":"crossref","first-page":"NP562","DOI":"10.1177\/1010539513480231","article-title":"Spectrum of hemoglobinopathies among the primitive tribes: a multicentric study in India","volume":"27","author":"Mohanty","year":"2015","journal-title":"Asia Pac J Public Health"},{"issue":"4","key":"2024121115353654000_hem2024000678C33","doi-asserted-by":"crossref","first-page":"316","DOI":"10.3109\/03630269.2012.691434","article-title":"Newborn screening shows a high incidence of sickle cell anemia in Central India","volume":"36","author":"Jain","year":"2012","journal-title":"Hemoglobin"},{"issue":"4","key":"2024121115353654000_hem2024000678C34","doi-asserted-by":"crossref","first-page":"323","DOI":"10.3109\/03630269.2012.697948","article-title":"Low fixed-dose hydroxyurea in severely affected Indian children with sickle cell disease","volume":"36","author":"Jain","year":"2012","journal-title":"Hemoglobin"},{"issue":"16-17","key":"2024121115353654000_hem2024000678C35","doi-asserted-by":"crossref","first-page":"1329","DOI":"10.1016\/j.clinbiochem.2010.08.006","article-title":"The effect of UGT1A1 promoter polymorphism on bilirubin response to hydroxyurea therapy in hemoglobinopathies","volume":"43","author":"Italia","year":"2010","journal-title":"Clin Biochem"},{"issue":"1","key":"2024121115353654000_hem2024000678C36","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/j.bcmd.2008.08.003","article-title":"Hydroxyurea in sickle cell disease\u2014a study of clinico\u2014pharmacological efficacy in the Indian haplotype","volume":"42","author":"Italia","year":"2009","journal-title":"Blood Cells Mol Dis"},{"issue":"4","key":"2024121115353654000_hem2024000678C37","doi-asserted-by":"crossref","first-page":"31","DOI":"10.3390\/ijns4040031","article-title":"Newborn screening for sickle cell disease: Indian experience","volume":"4","author":"Colah","year":"2018","journal-title":"Int J Neonatal Screen"},{"issue":"1","key":"2024121115353654000_hem2024000678C38","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1177\/0969141314557372","article-title":"Feasibility of a newborn screening and follow-up programme for sickle cell disease among South Gujarat (India) tribal populations","volume":"22","author":"Italia","year":"2015","journal-title":"J Med Screen"},{"issue":"12","key":"2024121115353654000_hem2024000678C39","doi-asserted-by":"crossref","first-page":"1791","DOI":"10.1515\/cclm-2014-0452","article-title":"Newborn screening for haemoglobinopathies by high performance liquid chromatography (HPLC): diagnostic utility of different approaches in resource-poor settings","volume":"52","author":"Upadhye","year":"2014","journal-title":"Clin Chem Lab Med"},{"issue":"3","key":"2024121115353654000_hem2024000678C40","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1007\/s12098-013-1218-1","article-title":"Newborn screening for sickle cell disease in India: the need for defining optimal clinical care","volume":"81","author":"Patel","year":"2014","journal-title":"Indian J Pediatr"},{"issue":"2","key":"2024121115353654000_hem2024000678C41","doi-asserted-by":"crossref","first-page":"101","DOI":"10.4103\/0971-6866.142841","article-title":"Guidelines for screening, diagnosis and management of hemoglobinopathies","volume":"20","author":"Ghosh","year":"2014","journal-title":"Indian J Hum Genet"},{"issue":"6","key":"2024121115353654000_hem2024000678C42","doi-asserted-by":"crossref","first-page":"747","DOI":"10.1007\/s12098-011-0682-8","article-title":"Neonatal screening of sickle cell anemia: a preliminary report","volume":"79","author":"Panigrahi","year":"2012","journal-title":"Indian J Pediatr"},{"issue":"3","key":"2024121115353654000_hem2024000678C43","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1016\/j.jiph.2017.08.013","article-title":"Comparative study of clinical presentation and hematological indices in hospitalized sickle cell patients with severe Plasmodium falciparum malaria","volume":"11","author":"Purohit","year":"2018","journal-title":"J Infect Public Health"},{"issue":"2","key":"2024121115353654000_hem2024000678C44","doi-asserted-by":"crossref","first-page":"294","DOI":"10.1007\/s12288-017-0869-x","article-title":"Beneficial effect of low fixed dose of hydroxyurea in vaso-occlusive crisis and transfusion requirements in adult HbSS patients: a prospective study in a tertiary care center","volume":"34","author":"Sethy","year":"2018","journal-title":"Indian J Hematol Blood Transfus"},{"issue":"2","key":"2024121115353654000_hem2024000678C45","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1007\/s12288-015-0542-1","article-title":"Hydroxyurea in sickle cell disease: our experience in Western India","volume":"32","author":"Deshpande","year":"2016","journal-title":"Indian J Hematol Blood Transfus"},{"issue":"6","key":"2024121115353654000_hem2024000678C46","doi-asserted-by":"crossref","first-page":"1017","DOI":"10.1002\/pbc.25391","article-title":"Low and fixed dose of hydroxyurea is effective and safe in patients with HbS\u03b2(+) thalassemia with IVS1-5(G\u2192C) mutation","volume":"62","author":"Dehury","year":"2015","journal-title":"Pediatr Blood Cancer"},{"issue":"10","key":"2024121115353654000_hem2024000678C47","doi-asserted-by":"crossref","first-page":"929","DOI":"10.1007\/s13312-013-0264-0","article-title":"Efficacy of fixed low dose hydroxyurea in Indian children with sickle cell anemia: a single centre experience","volume":"50","author":"Jain","year":"2013","journal-title":"Indian Pediatr"},{"issue":"5","key":"2024121115353654000_hem2024000678C48","doi-asserted-by":"crossref","first-page":"1825","DOI":"10.4103\/jfmpc.jfmpc_1490_23","article-title":"Morbidity pattern and impact of hydroxyurea therapy among sickle cell patients in Raipur district of Chhattisgarh","volume":"13","author":"Vaibhav","year":"2024","journal-title":"J Family Med Prim Care"},{"issue":"8","key":"2024121115353654000_hem2024000678C49","doi-asserted-by":"crossref","first-page":"1341","DOI":"10.1002\/pbc.25004","article-title":"The effect of hydroxyurea on compound heterozygotes for sickle cell-hemoglobin D-Punjab\u2014a single centre experience in eastern India","volume":"61","author":"Patel","year":"2014","journal-title":"Pediatr Blood Cancer"},{"issue":"5","key":"2024121115353654000_hem2024000678C50","doi-asserted-by":"crossref","first-page":"409","DOI":"10.3109\/03630269.2012.709897","article-title":"Low dose hydroxyurea is effective in reducing the incidence of painful crisis and frequency of blood transfusion in sickle cell anemia patients from eastern India","volume":"36","author":"Patel","year":"2012","journal-title":"Hemoglobin"},{"issue":"2","key":"2024121115353654000_hem2024000678C51","doi-asserted-by":"crossref","first-page":"299","DOI":"10.4103\/ijmr.ijmr_3447_21","article-title":"A systematic review on hydroxyurea therapy for sickle cell disease in India","volume":"156","author":"Pandey","year":"2022","journal-title":"Indian J Med Res"},{"issue":"6","key":"2024121115353654000_hem2024000678C52","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1016\/j.mjafi.2023.09.002","article-title":"Hematopoietic cell transplantation landscape in India","volume":"79","author":"Nair","year":"2023","journal-title":"Med J Armed Forces India"},{"issue":"4","key":"2024121115353654000_hem2024000678C53","doi-asserted-by":"crossref","first-page":"e28909","DOI":"10.1002\/pbc.28909","article-title":"Pretransplant myeloid and immune suppression, reduced toxicity conditioning with posttransplant cyclophosphamide: initial outcomes of novel approach for matched unrelated donor hematopoietic stem cell transplant for hemoglobinopathies","volume":"68","author":"Kharya","year":"2021","journal-title":"Pediatr Blood Cancer"},{"issue":"12","key":"2024121115353654000_hem2024000678C54","doi-asserted-by":"crossref","first-page":"1505","DOI":"10.1002\/ajh.26754","article-title":"Blood diseases in Africa: redressing unjust disparities is an urgent unmet need","volume":"97","author":"Makani","year":"2022","journal-title":"Am J Hematol"},{"issue":"10","key":"2024121115353654000_hem2024000678C55","doi-asserted-by":"crossref","first-page":"848","DOI":"10.3390\/cells13100848","article-title":"Current and future therapeutics for treating patients with sickle cell disease","volume":"13","author":"Barak","year":"2024","journal-title":"Cells"},{"issue":"746","key":"2024121115353654000_hem2024000678C56","doi-asserted-by":"crossref","first-page":"eadn1902","DOI":"10.1126\/scitranslmed.adn1902","article-title":"The translational gap for gene therapies in low- and middle-income countries","volume":"16","author":"Doxzen","year":"2024","journal-title":"Sci Transl Med"},{"issue":"1","key":"2024121115353654000_hem2024000678C57","doi-asserted-by":"crossref","first-page":"1794","DOI":"10.1038\/s41467-024-46036-x","article-title":"Development of pathophysiologically relevant models of sickle cell disease and \u03b2-thalassemia for therapeutic studies","volume":"15","author":"Gupta","year":"2024","journal-title":"Nat Commun"}],"container-title":["Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/ashpublications.org\/hematology\/article-pdf\/2024\/1\/1\/2346519\/1gupta.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/ashpublications.org\/hematology\/article-pdf\/2024\/1\/1\/2346519\/1gupta.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,12,11]],"date-time":"2024-12-11T19:38:37Z","timestamp":1733945917000},"score":1,"resource":{"primary":{"URL":"https:\/\/ashpublications.org\/hematology\/article\/2024\/1\/1\/526176\/Sickle-cell-disease-in-India-the-journey-and-hope"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,12,6]]},"references-count":57,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2024,12,6]]},"published-print":{"date-parts":[[2024,12,6]]}},"URL":"https:\/\/doi.org\/10.1182\/hematology.2024000678","relation":{},"ISSN":["1520-4391","1520-4383"],"issn-type":[{"value":"1520-4391","type":"print"},{"value":"1520-4383","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2024,12,6]]},"published":{"date-parts":[[2024,12,6]]}}}