Sickle cell anaemia

References

Key articles

Wethers DL. Sickle cell disease in childhood: part I. Laboratory diagnosis, pathophysiology and health maintenance. Am Fam Physician. 2000 Sep 1;62(5):1013-20, 1027-8.Full text  Abstract

DeBaun MR, Jordan LC, King AA, et al. American Society of Hematology 2020 guidelines for sickle cell disease: prevention, diagnosis, and treatment of cerebrovascular disease in children and adults. Blood Adv. 2020 Apr 28;4(8):1554-88.Full text  Abstract

National Heart, Lung, and Blood Institute. Evidence-based management of sickle cell disease: expert panel report, 2014. Sep 2014 [internet publication].Full text

Chou ST, Alsawas M, Fasano RM, et al. American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support. Blood Adv. 2020 Jan 28;4(2):327-55.Full text  Abstract

Trompeter S, Massey E, Robinson S, et al. Position paper on International Collaboration for Transfusion Medicine (ICTM) Guideline 'Red blood cell specifications for patients with hemoglobinopathies: a systematic review and guideline'. Br J Haematol. 2020 May;189(3):424-27.Full text  Abstract

Reference articles

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14. Kato GJ, Onyekwere OC, Gladwin MT. Pulmonary hypertension in sickle cell disease: relevance to children. Pediatr Hematol Oncol. 2007 Apr-May;24(3):159-70.Full text  Abstract

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27. DeBaun MR, Jordan LC, King AA, et al. American Society of Hematology 2020 guidelines for sickle cell disease: prevention, diagnosis, and treatment of cerebrovascular disease in children and adults. Blood Adv. 2020 Apr 28;4(8):1554-88.Full text  Abstract

28. Cherry MG, Greenhalgh J, Osipenko L, et al. The clinical effectiveness and cost-effectiveness of primary stroke prevention in children with sickle cell disease: a systematic review and economic evaluation. Health Technol Assess. 2012;16(43):1-129.Full text  Abstract

29. Alexandrov AV, Sloan MA, Tegeler CH, et al; American Society of Neuroimaging Practice Guidelines Committee. Practice standards for transcranial Doppler (TCD) ultrasound. Part II. Clinical indications and expected outcomes. J Neuroimaging. 2012 Jul;22(3):215-24. Abstract

30. Pecker LH, Sharma D, Nero A, et al. Knowledge gaps in reproductive and sexual health in girls and women with sickle cell disease. Br J Haematol. 2021 Sep;194(6):970-79.Full text  Abstract

31. Asnani MR, Quimby KR, Bennett NR, et al. Interventions for patients and caregivers to improve knowledge of sickle cell disease and recognition of its related complications. Cochrane Database Syst Rev. 2016 Oct 6;(10):CD011175.Full text  Abstract

32. National Heart, Lung, and Blood Institute. Evidence-based management of sickle cell disease: expert panel report, 2014. Sep 2014 [internet publication].Full text

33. Rankine-Mullings AE, Owusu-Ofori S. Prophylactic antibiotics for preventing pneumococcal infection in children with sickle cell disease. Cochrane Database Syst Rev. 2021 Mar 8;(3):CD003427.Full text  Abstract

34. Dekker LH, Fijnvandraat K, Brabin BJ, et al. Micronutrients and sickle cell disease, effects on growth, infection and vaso-occlusive crisis: a systematic review. Pediatr Blood Cancer. 2012 Aug;59(2):211-5. Abstract

35. Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease - life expectancy and risk factors for early death. N Engl J Med. 1994 Jun 9;330(23):1639-44.Full text  Abstract

36. Redwood AM, Williams EM, Desai P, et al. Climate and painful crisis of sickle-cell disease in Jamaica. BMJ. 1976 Jan 10;1(6001):66-8.Full text  Abstract

37. Mohan J, Marshall JM, Reid HL, et al. Peripheral vascular response to mild indirect cooling in patients with homozygous sickle cell (SS) disease and the frequency of painful crisis. Clin Sci (Lond). 1998 Feb;94(2):111-20. Abstract

38. Beutler E. Disorders of hemoglobin structure: sickle cell anemia and related abnormalities. In: Lichtman MA, Beutler E, Kaushansky K, et al, eds. Williams hematology. 7th ed. New York, NY: McGraw-Hill; 2006:667-700.

39. Charache S, Terrin ML, Moore RD, et al. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. N Engl J Med. 1995 May 18;332(20):1317-22.Full text  Abstract

40. Lanzkron S, Strouse JJ, Wilson R, et al. Systematic review: hydroxyurea for the treatment of adults with sickle cell disease. Ann Intern Med. 2008 Jun 17;148(12):939-55.Full text  Abstract

41. Segal JB, Strouse JJ, Beach MC, et al. Hydroxyurea for the treatment of sickle cell disease. Evid Rep Technol Assess (Full Rep). 2008 Mar;(165):1-95. Abstract

42. Hankins JS, Ware RE, Rogers ZR, et al. Long-term hydroxyurea therapy for infants with sickle cell anemia: the HUSOFT extension study. Blood. 2005 Oct 1;106(7):2269-75.Full text  Abstract

43. Scott JP, Hillery CA, Brown ER, et al. Hydroxyurea therapy in children severely affected with sickle cell disease. J Pediatr. 1996 Jun;128(6):820-8. Abstract

44. Wang WC, Helms RW, Lynn HS, et al. Effect of hydroxyurea on growth in children with sickle cell anemia: results of the HUG-KIDS Study. J Pediatr. 2002 Feb;140(2):225-9. Abstract

45. Wang WC, Ware RE, Miller ST, et al; BABY HUG investigators. Hydroxycarbamide in very young children with sickle-cell anaemia: a multicentre, randomised, controlled trial (BABY HUG). Lancet. 2011 May 14;377(9778):1663-72.Full text  Abstract

46. Zimmerman SA, Schultz WH, Davis JS, et al. Sustained long-term hematologic efficacy of hydroxyurea at maximum tolerated dose in children with sickle cell disease. Blood. 2004 Mar 15;103(6):2039-45.Full text  Abstract

47. Nevitt SJ, Jones AP, Howard J. Hydroxyurea (hydroxycarbamide) for sickle cell disease. Cochrane Database Syst Rev. 2017 Apr 20;(4):CD002202.Full text  Abstract

48. Davies S, Olujohungbe A. Hydroxyurea for sickle cell disease. Cochrane Database Syst Rev. 2001(2):CD002202. Abstract

49. Ware RE, Helms RW; SWiTCH Investigators. Stroke with transfusions changing to hydroxyurea (SWiTCH). Blood. 2012 Apr 26;119(17):3925-32.Full text  Abstract

50. Kinney TR, Helms RW, O'Branski EE, 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 Sep 1;94(5):1550-4.Full text  Abstract

51. Steinberg MH, Barton F, Castro O, et al. Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment. JAMA. 2003 Apr 2;289(13):1645-51.Full text  Abstract

52. Voskaridou E, Christoulas D, Bilalis A, et al. The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS). Blood. 2010 Mar 25;115(12):2354-63.Full text  Abstract

53. Steinberg MH, McCarthy WF, Castro O, et al. The risks and benefits of long-term use of hydroxyurea in sickle cell anemia: a 17.5 year follow-up. Am J Hematol. 2010 Jun;85(6):403-8.Full text  Abstract

54. Cieri-Hutcherson NE, Hutcherson TC, Conway-Habes EE, et al. Systematic review of i-glutamine for prevention of vaso-occlusive pain crisis in patients with sickle cell disease. Pharmacotherapy. 2019 Nov;39(11):1095-1104. Abstract

55. Niihara Y, Miller ST, Kanter J, et al. A phase 3 trial of L-glutamine in sickle cell disease. N Engl J Med. 2018 Jul 19;379(3):226-35. Abstract

56. Quinn CT. L-glutamine for sickle cell anemia: more questions than answers. Blood. 2018 Aug 16;132(7):689-93. Abstract

57. Ataga KI, Kutlar A, Kanter J, et al. Crizanlizumab for the prevention of pain crises in sickle cell disease. N Engl J Med. 2017 Feb 2;376(5):429-39.Full text  Abstract

58. Kutlar A, Kanter J, Liles DK, et al. Effect of crizanlizumab on pain crises in subgroups of patients with sickle cell disease: a SUSTAIN study analysis. Am J Hematol. 2019 Jan;94(1):55-61.Full text  Abstract

59. Han J, Saraf SL, Gordeuk VR. Systematic review of crizanlizumab: a new parenteral option to reduce vaso-occlusive pain crises in patients with sickle cell disease. Pharmacotherapy. 2020 Jun;40(6):535-43. Abstract

60. Çanak B, Eşkazan AE. Voxelotor: a new kid on the block in the treatment of sickle cell disease. Br J Clin Pharmacol. 14 Feb 2022 [internet publication].Full text  Abstract

61. Vichinsky E, Hoppe CC, Ataga KI, et al. A phase 3 randomized trial of voxelotor in sickle cell disease. N Engl J Med. 2019 Aug 8;381(6):509-19.Full text  Abstract

62. Han J, Saraf SL, Gordeuk VR. Systematic review of voxelotor: a first-in-class sickle hemoglobin polymerization inhibitor for management of sickle cell disease. Pharmacotherapy. 2020 Jun;40(6):525-34. Abstract

63. U.S. National Library of medicine. Clinical trials. Study to evaluate the effect of GBT440 on TCD in pediatrics with sickle cell disease (HOPE Kids 2). Dec 2020 [internet publication].Full text

64. Vichinsky EP, Haberkern CM, Neumayr L, et al; The Preoperative Transfusion in Sickle Cell Disease Study Group. A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease. N Engl J Med. 1995 Jul 27;333(4):206-13.Full text  Abstract

65. Estcourt LJ, Kimber C, Trivella M, et al. Preoperative blood transfusions for sickle cell disease. Cochrane Database Syst Rev. 2020 Jul 2;(7):CD003149.Full text  Abstract

66. Howard J, Malfroy M, Llewelyn C, et al. The Transfusion Alternatives Preoperatively in Sickle Cell Disease (TAPS) study: a randomised, controlled, multicentre clinical trial. Lancet. 2013 Mar 16;381(9870):930-8. Abstract

67. Lee MT, Piomelli S, Granger S, et al. Stroke Prevention Trial in Sickle Cell Anemia (STOP): extended follow-up and final results. Blood. 2006 Aug 1;108(3):847-52.Full text  Abstract

68. Adams RJ, Brambilla D. Discontinuing prophylactic transfusions used to prevent stroke in sickle cell disease. N Engl J Med. 2005 Dec 29;353(26):2769-78.Full text  Abstract

69. Compernolle V, Chou ST, Tanael S, et al. Red blood cell specifications for patients with hemoglobinopathies: a systematic review and guideline. Transfusion. 2018 Jun;58(6):1555-66. Abstract

70. Chou ST, Alsawas M, Fasano RM, et al. American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support. Blood Adv. 2020 Jan 28;4(2):327-55.Full text  Abstract

71. Trompeter S, Massey E, Robinson S, et al. Position paper on International Collaboration for Transfusion Medicine (ICTM) Guideline 'Red blood cell specifications for patients with hemoglobinopathies: a systematic review and guideline'. Br J Haematol. 2020 May;189(3):424-27.Full text  Abstract

72. Fasano RM, Meyer EK, Branscomb J, et al. Impact of red blood cell antigen matching on alloimmunization and transfusion complications in patients with sickle cell disease: a systematic review. Transfus Med Rev. 2019 Jan;33(1):12-23. Abstract

73. Oteng-Ntim E, Pavord S, Howard R, et al. Management of sickle cell disease in pregnancy. A British Society for Haematology Guideline. Br J Haematol. 2021 Sep;194(6):980-95.Full text  Abstract

74. Boafor TK, Olayemi E, Galadanci N, et al. Pregnancy outcomes in women with sickle-cell disease in low and high income countries: a systematic review and meta-analysis. BJOG. 2016 Apr;123(5):691-8. Abstract

75. Malinowski AK, Shehata N, D'Souza R, et al. Prophylactic transfusion for pregnant women with sickle cell disease: a systematic review and meta-analysis. Blood. 2015 Nov 19;126(21):2424-35.Full text  Abstract

76. Brandow AM, Carroll CP, Creary S, et al. American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain. Blood Adv. 2020 Jun 23;4(12):2656-2701.Full text  Abstract

77. Jacobson SJ, Kopecky EA, Joshi P, et al. Randomised trial of oral morphine for painful episodes of sickle-cell disease in children. Lancet. 1997 Nov 8;350(9088):1358-61. Abstract

78. Cooper TE, Hambleton IR, Ballas SK, et al. Pharmacological interventions for painful sickle cell vaso-occlusive crises in adults. Cochrane Database Syst Rev. 2019 Nov 14;2019(11):CD012187.Full text  Abstract

79. Aboursheid T, Albaroudi O, Alahdab F. Inhaled nitric oxide for treating pain crises in people with sickle cell disease. Cochrane Database Syst Rev. 2019 Oct 11;(10):CD011808.Full text  Abstract

80. Dampier CD, Wager CG, Harrison R, et al; Investigators of the Sickle Cell Disease Clinical Research Network (SCDCRN). Impact of PCA strategies on pain intensity and functional assessment measures in adults with sickle cell disease during hospitalized vaso-occlusive episodes. Am J Hematol. 2012 Oct;87(10):E71-4.Full text  Abstract

81. Vichinsky EP, Neumayr LD, Earles AN, 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 Jun 22;342(25):1855-65.Full text  Abstract

82. Dolatkhah R, Dastgiri S. Blood transfusions for treating acute chest syndrome in people with sickle cell disease. Cochrane Database Syst Rev. 2020 Jan 16;(1):CD007843.Full text  Abstract

83. Bundy DG, Richardson TE, Hall M, et al. Association of guideline-adherent antibiotic treatment with readmission of children with sickle cell disease hospitalized with acute chest syndrome. JAMA Pediatr. 2017 Nov 1;171(11):1090-9.Full text  Abstract

84. Kanter J, Liem RI, Bernaudin F, et al. American Society of Hematology 2021 guidelines for sickle cell disease: stem cell transplantation. Blood Adv. 2021 Sep 28;5(18):3668-89.Full text  Abstract

85. Oringanje C, Nemecek E, Oniyangi O. Hematopoietic stem cell transplantation for people with sickle cell disease. Cochrane Database Syst Rev. 2020 Jul 3;(7):CD007001.Full text  Abstract

86. Shenoy S, Angelucci E, Arnold SD, et al. Current results and future research priorities in late effects after hematopoietic stem cell transplantation for children with sickle cell disease and thalassemia: a consensus statement from the Second Pediatric Blood and Marrow Transplant Consortium International Conference on Late Effects after Pediatric Hematopoietic Stem Cell Transplantation. Biol Blood Marrow Transplant. 2017 Apr;23(4):552-61.Full text  Abstract

87. Smith WR, Penberthy LT, Bovbjerg VE, et al. Daily assessment of pain in adults with sickle cell disease. Ann Intern Med. 2008 Jan 15;148(2):94-101. Abstract

88. Anie KA, Green J. Psychological therapies for sickle cell disease and pain. Cochrane Database Syst Rev. 2015 May 8;(5):CD001916.Full text  Abstract

89. Webster J, Osborne S, Rickard CM, et al. Clinically-indicated replacement versus routine replacement of peripheral venous catheters. Cochrane Database Syst Rev. 2019 Jan 23;(1):CD007798.Full text  Abstract

90. Martí-Carvajal AJ, Solà I, Agreda-Pérez LH. Treatment for avascular necrosis of bone in people with sickle cell disease. Cochrane Database Syst Rev. 2019 Dec 5;(12):CD004344.Full text  Abstract

91. US Food and Drug Administration. FDA drug safety communication: FDA restricts use of prescription codeine pain and cough medicines and tramadol pain medicines in children; recommends against use in breastfeeding women. Apr 2017 [internet publication].Full text

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95. Misra H, Bainbridge J, Berryman J, et al. A Phase Ib open label, randomized, safety study of SANGUINATE™ in patients with sickle cell anemia. Rev Bras Hematol Hemoter. 2017 Jan-Mar;39(1):20-7.Full text  Abstract

96. Valentino J, Misra H, Lopez L, Paulino GM. Use of pegylated-carboxyhemoglobin bovine for the treatment of sickle cell disease associated leg ulcers: results from a phase 2 safety study. Presented at: Haematologica. 22th congress of the european hematology association. Spain, 2017.Full text

97. McArthur JG, Svenstrup N, Chen C, et al. A novel, highly potent and selective phosphodiesterase-9 inhibitor for the treatment of sickle cell disease. Haematologica. 2020 Mar; 105(3):623–31.Full text  Abstract

98. U.S. National library of medicine. Clinical trials. A study of IMR-687 in subjects with sickle cell disease. Jul 2020 [internet publication].Full text

99. ClinicalTrials.gov. A study evaluating the efficacy and safety of Mitapivat (AG-348) in participants with sickle cell disease. ClinicalTrials.gov Identifier: NCT05031780. Mar 2022 [internet publication].Full text

100. Rab MAE, Van Oirschot BA, Kosinski PA, et al. AG-348 (Mitapivat), an allosteric activator of red blood cell pyruvate kinase, increases enzymatic activity, protein stability, and ATP levels over a broad range of PKLR genotypes. Haematologica. 2021 Jan 1;106(1):238-49.Full text  Abstract

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107. Estcourt LJ, Kohli R, Hopewell S, et al. Blood transfusion for preventing primary and secondary stroke in people with sickle cell disease. Cochrane Database Syst Rev. 2020 Jul 27;(7):CD003146.Full text  Abstract

108. Hulbert ML, McKinstry RC, Lacey JL, et al. Silent cerebral infarcts occur despite regular blood transfusion therapy after first strokes in children with sickle cell disease. Blood. 2011 Jan 20;117(3):772-9.Full text  Abstract

109. Estcourt LJ, Kimber C, Hopewell S, et al. Interventions for preventing silent cerebral infarcts in people with sickle cell disease. Cochrane Database Syst Rev. 2020 Apr 6;(4):CD012389.Full text  Abstract

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116. Bonatsos G, Birbas K, Toutouzas K, et al. Laparoscopic cholecystectomy in adults with sickle cell disease. Surg Endosc. 2001 Aug;15(8):816-9. Abstract

117. Owusu-Ofori S, Remmington T. Splenectomy versus conservative management for acute sequestration crises in people with sickle cell disease. Cochrane Database Syst Rev. 2017 Nov 7;(11):CD003425.Full text  Abstract

118. Oligbu G, Fallaha M, Pay L, et al. Risk of invasive pneumococcal disease in children with sickle cell disease in the era of conjugate vaccines: a systematic review of the literature. Br J Haematol. 2019 May;185(4):743-751.Full text  Abstract

119. Claster S, Vichinsky EP. Managing sickle cell disease. BMJ. 2003 Nov 15;327(7424):1151-5.Full text  Abstract

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122. Myint KT, Sahoo S, Thein AW, et al. Laser therapy for retinopathy in sickle cell disease. Cochrane Database Syst Rev. 2015 Oct 9;(10):CD010790.Full text  Abstract

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124. Castro O, Gladwin MT. Pulmonary hypertension in sickle cell disease: mechanisms, diagnosis, and management. Hematol Oncol Clin North Am. 2005 Oct;19(5):881-96. Abstract

125. Klings ES, Machado RF, Barst RJ, et al.; American Thoracic Society Ad Hoc Committee on Pulmonary Hypertension of Sickle Cell Disease. An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease. Am J Respir Crit Care Med. 2014 Mar 15;189(6):727-40. Abstract

126. Machado RF, Martyr S, Kato GJ, et al. Sildenafil therapy in patients with sickle cell disease and pulmonary hypertension. Br J Haematol. 2005 Aug;130(3):445-53.Full text  Abstract

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