Sickle Cell Disease in Sub-Saharan Africa

Hematology/Oncology Clinics of North America

Volumn 30, Issue 2, 2016, Pages 343-358

  Williams, Thomas N ^a,b  

^a ST MARY S HOSPITAL   (United Kingdom)

^b KEMRI WELLCOME TRUST RESEARCH PROGRAMME   (Kenya)

Author keywords

Bacteremia; Children; Public health; Sickle cell disease; Sub Saharan Africa

Indexed keywords

ERYTHROCYTE MEMBRANE PROTEIN 1; HEMOGLOBIN S; HEMOGLOBIN BETA CHAIN;

AFRICA SOUTH OF THE SAHARA; ALLOGENEIC HEMATOPOIETIC STEM CELL TRANSPLANTATION; APPLIED RESEARCH; BACTEREMIA; BASIC RESEARCH; CAUSE OF DEATH; DISEASE SURVEILLANCE; EARLY DIAGNOSIS; GEOGRAPHIC DISTRIBUTION; HAPLOTYPE; HEALTH CARE AVAILABILITY; HIGH RISK POPULATION; HISTORY OF MEDICINE; HUMAN; INCIDENCE; MALARIA; MORTALITY; MUTATIONAL ANALYSIS; NEWBORN SCREENING; NONHUMAN; PREVALENCE; PRIORITY JOURNAL; RESOURCE ALLOCATION; REVIEW; SICKLE CELL ANEMIA; AGE; ANEMIA, SICKLE CELL; COMORBIDITY; COST OF ILLNESS; GENETICS; GLOBAL HEALTH; HEALTH CARE DELIVERY; MUTATION;

AFRICA SOUTH OF THE SAHARA; AGE FACTORS; ANEMIA, SICKLE CELL; BETA-GLOBINS; CAUSE OF DEATH; COMORBIDITY; COST OF ILLNESS; DELIVERY OF HEALTH CARE; GLOBAL HEALTH; HUMANS; MUTATION;

EID: 84962074404     PISSN: 08898588     EISSN: 15581977     Source Type: Journal    
DOI: 10.1016/j.hoc.2015.11.005     Document Type: Review

References (98)

1. Murayama M. Structure of sickle cell hemoglobin and molecular mechanism of the sickling phenomenon. Clin Chem 1967, 13(7):578-588.
2. Rees D.C., Williams T.N., Gladwin M.T. Sickle-cell disease. Lancet 2010, 376(9757):2018-2031.
3. Modell B., Darlison M. Global epidemiology of haemoglobin disorders and derived service indicators. Bull World Health Organ 2008, 86(6):480-487.
4. Weatherall D.J., Clegg J.B. The thalassaemia syndromes 2002, Blackwell Scientific Publications, Oxford (United Kingdom). 3rd edition.
5. Kulozik A.E., Wainscoat J.S., Serjeant G.R., et al. Geographical survey of beta S-globin gene haplotypes: evidence for an independent Asian origin of the sickle-cell mutation. Am J Hum Genet 1986, 39(2):239-244.
6. Flint J., Harding R.M., Boyce A.J., et al. The population genetics of the haemoglobinopathies. Baillieres Clin Haematol 1998, 11(1):1-51.
7. Piel F.B., Patil A.P., Howes R.E., et al. Global distribution of the sickle cell gene and geographical confirmation of the malaria hypothesis. Nat Commun 2010, 1(8):104.
8. Allison A.C. Protection afforded by sickle cell trait against subtertian malarial infection. Br Med J 1954, 1:290-295.
9. Williams T.N. Human red blood cell polymorphisms and malaria. Curr Opin Microbiol 2006, 9(4):388-394.
10. Taylor S.M., Parobek C.M., Fairhurst R.M. Haemoglobinopathies and the clinical epidemiology of malaria: a systematic review and meta-analysis. Lancet Infect Dis 2012, 12(6):457-468.
11. The Malaria Genomic Epidemiology Network Reappraisal of known malaria resistance loci in a large multicenter study. Nat Genet 2014, 46(11):1197-1204.
12. Kreuels B., Kreuzberg C., Kobbe R., et al. Differing effects of HbS and HbC traits on uncomplicated falciparum malaria, anemia, and child growth. Blood 2010, 115(22):4551-4558.
13. Nyakeriga A.M., Troye-Blomberg M., Chemtai A.K., et al. Malaria and nutritional status in children living on the coast of Kenya. Am J Clin Nutr 2004, 80(6):1604-1610.
14. Scott J.A., Berkley J.A., Mwangi I., et al. Relation between falciparum malaria and bacteraemia in Kenyan children: a population-based, case-control study and a longitudinal study. Lancet 2011, 378(9799):1316-1323.
15. Eeckels R., Gatti F., Renoirte A.M. Abnormal distribution of haemoglobin genotypes in Negro children with severe bacterial infections. Nature 1967, 216(113):382.
16. Friedman M.J. Erythrocytic mechanism of sickle cell resistance to malaria. Proc Natl Acad Sci U S A 1978, 75(4):1994-1997.
17. Friedman M.J., Roth E.F., Nagel R.L., et al. The role of hemoglobins C, S, and Nbalt in the inhibition of malaria parasite development in vitro. Am J Trop Med Hyg 1979, 28(5):777-780.
18. Pasvol G., Weatherall D.J., Wilson R.J. Cellular mechanism for the protective effect of haemoglobin S against P. falciparum malaria. Nature 1978, 274(5672):701-703.
19. Fairhurst R.M., Fujioka H., Hayton K., et al. Aberrant development of Plasmodium falciparum in hemoglobin CC red cells: implications for the malaria protective effect of the homozygous state. Blood 2003, 101(8):3309-3315.
20. Cholera R., Brittain N.J., Gillrie M.R., et al. Impaired cytoadherence of Plasmodium falciparum-infected erythrocytes containing sickle hemoglobin. Proc Natl Acad Sci U S A 2008, 105(3):991-996.
21. Fairhurst R.M., Baruch D.I., Brittain N.J., et al. Abnormal display of PfEMP-1 on erythrocytes carrying haemoglobin C may protect against malaria. Nature 2005, 435(7045):1117-1121.
22. Ayi K., Turrini F., Piga A., et al. Enhanced phagocytosis of ring-parasitized mutant erythrocytes. A common mechanism that may explain protection against falciparum malaria in sickle trait and beta-thalassemia trait. Blood 2004, 104:3364-3371.
23. Luzzatto L., Nwachuku-Jarrett E.S., Reddy S. Increased sickling of parasitised erythrocytes as mechanism of resistance against malaria in the sickle-cell trait. Lancet 1970, 1(7642):319-321.
24. Roth E.F., Friedman M., Ueda Y., et al. Sickling rates of human AS red cells infected in vitro with Plasmodium falciparum malaria. Science 1978, 202(4368):650-652.
25. Shear H.L., Roth E.F., Fabry M.E., et al. Transgenic mice expressing human sickle hemoglobin are partially resistant to rodent malaria. Blood 1993, 81(1):222-226.
26. Herrick J.B. Peculiar elongated and sickle-shaped red blood corpuscles in a case of severe anemia. Arch Intern Med 1910, 6(5):517-521.
27. Lehmann H., Raper A.B. Maintenance of high sickling rate in an African community. Br Med J 1956, 2(4988):333-336.
28. Grosse S.D., Odame I., Atrash H.K., et al. Sickle cell disease in Africa: a neglected cause of early child mortality. Am J Prev Med 2011, 41(6 Suppl 4):S398-405.
29. Telfer P., Coen P., Chakravorty S., et al. Clinical outcomes in children with sickle cell disease living in England: a neonatal cohort in East London. Haematologica 2007, 92(7):905-912.
30. Quinn C.T., Rogers Z.R., McCavit T.L., et al. Improved survival of children and adolescents with sickle cell disease. Blood 2010, 115(17):3447-3452.
31. Wierenga K.J., Hambleton I.R., Lewis N.A. Survival estimates for patients with homozygous sickle-cell disease in Jamaica: a clinic-based population study. Lancet 2001, 357(9257):680-683.
32. Piel F.B., Patil A.P., Howes R.E., et al. Global epidemiology of sickle haemoglobin in neonates: a contemporary geostatistical model-based map and population estimates. Lancet 2012, 381(9861):142-151.
33. +-thalassemia and the sickle cell trait. Nat Genet 2005, 37:1253-1257.
34. Pasvol G., Weatherall D., Wilson R.J.M. The increased susceptibility of young red cells to invasion by the malaria parasite Plasmodium falciparum. Br J Haematol 1980, 45:285-295.
35. Lamikanra A.A., Brown D., Potocnik A., et al. Malarial anemia: of mice and men. Blood 2007, 110(1):18-28.
36. Trowell H.C., Raper A.B., Welbourn H.F. The natural history of homozygous sickle-cell anaemia in Central Africa. Q J Med 1957, 26(104):401-422.
37. Lambotte-Legrand J., Lambotte-Legrand C. Le prognostic de l'anemie drepanocytaire au Congo Belge (a propos de 300 case et de 150 deces). Ann Soc Belg Med Trop 1955, 35:53-57.
38. Adeloye A., Luzzatto L., Edington G.M. Severe malarial infection in a patient with sickle-cell anaemia. Br Med J 1971, 2(759):445-446.
39. Konotey-Ahulu F.I. Malaria and sickle-cell disease. Br Med J 1971, 2(763):710-711.
40. Seymour A. Malaria and sickle cell disease. Br Med J 1971, 2(763):711.
41. Pichanick A.M. Severe malarial infection. Br Med J 1971, 3(766):114.
42. Ambe J.P., Fatunde J.O., Sodeinde O.O. Associated morbidities in children with sickle-cell anaemia presenting with severe anaemia in a malarious area. Trop Doct 2001, 31(1):26-27.
43. Ibidapo M.O., Akinyanju O.O. Acute sickle cell syndromes in Nigerian adults. Clin Lab Haematol 2000, 22(3):151-155.
44. Okuonghae H.O., Nwankwo M.U., Offor E. Malarial parasitaemia in febrile children with sickle cell anaemia. J Trop Pediatr 1992, 38(2):83-85.
45. Aidoo M., Terlouw D.J., Kolczak M.S., et al. Protective effects of the sickle cell gene against malaria morbidity and mortality. Lancet 2002, 359(9314):1311-1312.
46. Oniyangi O., Omari A.A. Malaria chemoprophylaxis in sickle cell disease. Cochrane Database Syst Rev 2006, (4). CD003489.
47. Wollstein M., Kreidel K.V. Sickle cell anemia. Am J Dis Child 1928, 36:998-1011.
48. Robinson M.G., Watson R.J. Pneumococcal meningitis in sickle-cell anemia. N Engl J Med 1966, 274(18):1006-1008.
49. Barrett-Connor E. Bacterial infection and sickle cell anemia. An analysis of 250 infections in 166 patients and a review of the literature. Medicine (Baltimore) 1971, 50(2):97-112.
50. Lobel J.S., Bove K.E. Clinicopathologic characteristics of septicemia in sickle cell disease. Am J Dis Child 1982, 136(6):543-547.
51. Powars D., Overturf G., Turner E. Is there an increased risk of Haemophilus influenzae septicemia in children with sickle cell anemia?. Pediatrics 1983, 71(6):927-931.
52. Diggs L.W. Bone and joint lesions in sickle-cell disease. Clin Orthop 1967, 52:119-143.
53. Akinyanju O., Johnson A.O. Acute illness in Nigerian children with sickle cell anaemia. Ann Trop Paediatr 1987, 7(3):181-186.
54. Okuonghae H.O., Nwankwo M.U., Offor E.C. Pattern of bacteraemia in febrile children with sickle cell anaemia. Ann Trop Paediatr 1993, 13(1):55-64.
55. Akuse R.M. Variation in the pattern of bacterial infection in patients with sickle cell disease requiring admission. J Trop Pediatr 1996, 42(6):318-323.
56. Aken'ova Y.A., Bakare R.A., Okunade M.A. Septicaemia in sickle cell anaemia patients: the Ibadan experience. Cent Afr J Med 1998, 44(4):102-104.
57. Kizito M.E., Mworozi E., Ndugwa C., et al. Bacteraemia in homozygous sickle cell disease in Africa: is pneumococcal prophylaxis justified?. Arch Dis Child 2007, 92(1):21-23.
58. Makani J., Mgaya J., Balandya E., et al. Bacteraemia in sickle cell anaemia is associated with low haemoglobin: a report of 890 admissions to a tertiary hospital in Tanzania. Br J Haematol 2015, [Epub ahead of print].
59. Serjeant G.R. Mortality from sickle cell disease in Africa. BMJ 2005, 330(7489):432-433.
60. Williams T.N., Uyoga S., Macharia A., et al. Bacteraemia in Kenyan children with sickle-cell anaemia: a retrospective cohort and case-control study. Lancet 2009, 374(9698):1364-1370.
61. Ramakrishnan M., Moisi J.C., Klugman K.P., et al. Increased risk of invasive bacterial infections in African people with sickle-cell disease: a systematic review and meta-analysis. Lancet Infect Dis 2010, 10(5):329-337.
62. United Nations press office. Press conference on raising awareness of sickle-cell anaemia. 2009. Available at: . Accessed December 15, 2015. http://www.un.org/News/briefings/docs/2009/090619_Anaemia.doc.htm.
63. World Health Organization Regional Office for Africa. Sickle-cell disease: a strategy for the WHO African Region. Report of the Regional Director. AFR/RC60/8, 22 June 2010. 2010.
64. Tshilolo L., Aissi L.M., Lukusa D., et al. Neonatal screening for sickle cell anaemia in the Democratic Republic of the Congo: experience from a pioneer project on 31 204 newborns. J Clin Pathol 2009, 62(1):35-38.
65. Kafando E., Nacoulma E., Ouattara Y., et al. Neonatal haemoglobinopathy screening in Burkina Faso. J Clin Pathol 2009, 62(1):39-41.
66. McGann P.T., Ferris M.G., Ramamurthy U., et al. A prospective newborn screening and treatment program for sickle cell anemia in Luanda, Angola. Am J Hematol 2013, 88(12):984-989.
67. Marsh V.M., Kamuya D.M., Molyneux S.S. 'All her children are born that way': gendered experiences of stigma in families affected by sickle cell disorder in rural Kenya. Ethn Health 2011, 16(4-5):343-359.
68. Assimadi J.K., Gbadoe A.D., Nyadanu M. The impact on families of sickle cell disease in Togo. Arch Pediatr 2000, 7(6):615-620. [in French].
69. Bamisaiye A., Bakare C.G., Olatawura M.O. Some social-psychologic dimensions of sickle cell anemia among Nigerians. Clin Pediatr (Phila) 1974, 13(1):56-59.
70. Makani J., Soka D., Rwezaula S., et al. Health policy for sickle cell disease in Africa: experience from Tanzania on interventions to reduce under-five mortality. Trop Med Int Health 2015, 20(2):184-187.
71. Dzik W.S., Kyeyune D., Otekat G., et al. Transfusion medicine in sub-Saharan Africa: conference summary. Transfus Med Rev 2015, 29(3):195-204.
72. Ohene-Frempong K., Weiner S.J., Sleeper L.A., et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood 1998, 91(1):288-294.
73. Lukoo R.N., Ngiyulu R.M., Mananga G.L., et al. Depression in children suffering from sickle cell anemia. J Pediatr Hematol Oncol 2015, 37(1):20-24.
74. Ohaeri J.U., Shokunbi W.A., Akinlade K.S., et al. The psychosocial problems of sickle cell disease sufferers and their methods of coping. Soc Sci Med 1995, 40(7):955-960.
75. Piel F.B., Hay S.I., Gupta S., et al. Global burden of sickle cell anaemia in children under five, 2010-2050: modelling based on demographics, excess mortality, and interventions. PLoS Med 2013, 10(7):e1001484.
76. Piel F.B., Tatem A.J., Huang Z., et al. Global migration and the changing distribution of sickle haemoglobin: a quantitative study of temporal trends between 1960 and 2000. Lancet Glob Health 2014, 2(2):e80-e89.
77. Charache S., Terrin M.L., Moore R.D., et al. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. N Engl J Med 1995, 332(20):1317-1322.
78. Kinney T.R., Helms R.W., O'Branski E.E., et al. Safety of hydroxyurea in children with sickle cell anemia: results of the HUG-KIDS study, a phase I/II trial. Pediatric Hydroxyurea Group. Blood 1999, 94(5):1550-1554.
79. Wang W.C., Ware R.E., Miller S.T., et al. Hydroxycarbamide in very young children with sickle-cell anaemia: a multicentre, randomised, controlled trial (BABY HUG). Lancet 2011, 377(9778):1663-1672.
80. Ware R.E., Despotovic J.M., Mortier N.A., et al. Pharmacokinetics, pharmacodynamics, and pharmacogenetics of hydroxyurea treatment for children with sickle cell anemia. Blood 2011, 118(18):4985-4991.
81. Platt O.S., Orkin S.H., Dover G., et al. Hydroxyurea enhances fetal hemoglobin production in sickle cell anemia. J Clin Invest 1984, 74(2):652-656.
82. Lewis W.H., Wright J.A. Altered ribonucleotide reductase activity in mammalian tissue culture cells resistant to hydroxyurea. Biochem Biophys Res Commun 1974, 60(3):926-933.
83. Ware R.E. How I use hydroxyurea to treat young patients with sickle cell anemia. Blood 2010, 115(26):5300-5311.
84. Mulaku M., Opiyo N., Karumbi J., et al. Evidence review of hydroxyurea for the prevention of sickle cell complications in low-income countries. Arch Dis Child 2013, 98(11):908-914.
85. Galadanci N.A., Abdullahi S.U., Tabari M.A., et al. Primary stroke prevention in Nigerian children with sickle cell disease (SPIN): challenges of conducting a feasibility trial. Pediatr Blood Cancer 2015, 62(3):395-401.
86. Available at: . Accessed July 23, 2015. https://clinicaltrials.gov/ct2/show/record/NCT01966731.
87. Adams R.J., McKie V.C., Hsu L., et al. Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. N Engl J Med 1998, 339(1):5-11.
88. Adams R.J., Brambilla D., Optimizing Primary Stroke Prevention in Sickle Cell Anemia (STOP 2) Trial Investigators Discontinuing prophylactic transfusions used to prevent stroke in sickle cell disease. N Engl J Med 2005, 353(26):2769-2778.
89. Vichinsky E.P., Neumayr L.D., Earles A.N., et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med 2000, 342(25):1855-1865.
90. Miller S.T., Wright E., Abboud M., et al. Impact of chronic transfusion on incidence of pain and acute chest syndrome during the Stroke Prevention Trial (STOP) in sickle-cell anemia. J Pediatr 2001, 139(6):785-789.
91. Alvarez O., Yovetich N.A., Scott J.P., et al. Pain and other non-neurological adverse events in children with sickle cell anemia and previous stroke who received hydroxyurea and phlebotomy or chronic transfusions and chelation: results from the SWiTCH clinical trial. Am J Hematol 2013, 88(11):932-938.
92. Chou S.T. Transfusion therapy for sickle cell disease: a balancing act. Hematol Am Soc Hematol Educ Program 2013, 2013:439-446.
93. Wayne A.S., Schoenike S.E., Pegelow C.H. Financial analysis of chronic transfusion for stroke prevention in sickle cell disease. Blood 2000, 96(7):2369-2372.
94. Wang W.C., Oyeku S.O., Luo Z., et al. Hydroxyurea is associated with lower costs of care of young children with sickle cell anemia. Pediatrics 2013, 132(4):677-683.
95. Locatelli F., Pagliara D. Allogeneic hematopoietic stem cell transplantation in children with sickle cell disease. Pediatr Blood Cancer 2012, 59(2):372-376.
96. Chakravorty S., Williams T.N. Sickle cell disease: a neglected chronic disease of increasing global health importance. Arch Dis Child 2015, 100(1):48-53.
97. Dallas M.H., Triplett B., Shook D.R., et al. Long-term outcome and evaluation of organ function in pediatric patients undergoing haploidentical and matched related hematopoietic cell transplantation for sickle cell disease. Biol Blood Marrow Transplant 2013, 19(5):820-830.
98. Pule G., Wonkam A. Treatment for sickle cell disease in Africa: should we invest in haematopoietic stem cell transplantation?. Pan Afr Med J 2014, 18:46.