Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Red blood cell transfusion in sickle cell disease

Michael R DeBaun, MD, MPH
Elliott P Vichinsky, MD
Section Editors
Stanley L Schrier, MD
Donald H Mahoney, Jr, MD
Deputy Editor
Jennifer S Tirnauer, MD


Individuals with sickle cell disease (SCD) have chronic anemia that can worsen abruptly (eg, from splenic sequestration or transient red cell aplasia), and they are at risk of vaso-occlusive events (eg, stroke) due to the high concentration of sickle hemoglobin (HgbS) associated with their condition. Transfusion of red blood cells (RBCs) can be life-saving in these settings.

However, transfusion carries risks, many of which are greater in individuals with SCD than in the general population. The approach to transfusion in SCD must balance these benefits and risks, both in decisions regarding when to transfuse and in the practical aspects of how transfusions are administered.

Here we discuss our approach to transfusion and transfusion complications in SCD. The clinical manifestations of SCD, other aspects of SCD management, and more extensive discussions of transfusion-related excessive iron stores are presented separately:

Clinical manifestations (overview) – (see "Overview of the clinical manifestations of sickle cell disease")

Management (overview) – (see "Overview of the management and prognosis of sickle cell disease")

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Oct 2017. | This topic last updated: Jun 22, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Wayne AS, Kevy SV, Nathan DG. Transfusion management of sickle cell disease. Blood 1993; 81:1109.
  2. Davies SC, Roberts-Harewood M. Blood transfusion in sickle cell disease. Blood Rev 1997; 11:57.
  3. Nifong TP, Domen RE. Oxygen saturation and hemoglobin A content in patients with sickle cell disease undergoing erythrocytapheresis. Ther Apher 2002; 6:390.
  4. Uchida K, Rackoff WR, Ohene-Frempong K, et al. Effect of erythrocytapheresis on arterial oxygen saturation and hemoglobin oxygen affinity in patients with sickle cell disease. Am J Hematol 1998; 59:5.
  5. Josephson CD, Su LL, Hillyer KL, Hillyer CD. Transfusion in the patient with sickle cell disease: a critical review of the literature and transfusion guidelines. Transfus Med Rev 2007; 21:118.
  6. Quirolo K. How do I transfuse patients with sickle cell disease? Transfusion 2010; 50:1881.
  7. The management of sickle cell disease. National Institutes of Health; National Heart, Lung, and Blood Institute, Division of Blood Diseases and Resources. NIH publication 04-2117, revised 2004. This reference is also available for downloading or purchase at: www.nhlbi.nih.gov/health/prof/blood/sickle/ (Accessed on June 25, 2009).
  8. Dowling MM, Quinn CT, Plumb P, et al. Acute silent cerebral ischemia and infarction during acute anemia in children with and without sickle cell disease. Blood 2012; 120:3891.
  9. Goldstein AR, Anderson MJ, Serjeant GR. Parvovirus associated aplastic crisis in homozygous sickle cell disease. Arch Dis Child 1987; 62:585.
  10. Wierenga KJ, Pattison JR, Brink N, et al. Glomerulonephritis after human parvovirus infection in homozygous sickle-cell disease. Lancet 1995; 346:475.
  11. Quek L, Sharpe C, Dutt N, et al. Acute human parvovirus B19 infection and nephrotic syndrome in patients with sickle cell disease. Br J Haematol 2010; 149:289.
  12. Hassell KL, Eckman JR, Lane PA. Acute multiorgan failure syndrome: a potentially catastrophic complication of severe sickle cell pain episodes. Am J Med 1994; 96:155.
  13. Green M, Hall RJ, Huntsman RG, et al. Sickle cell crisis treated by exchange transfusion. Treatment of two patients with heterozygous sickle cell syndrome. JAMA 1975; 231:948.
  14. Miller ST, 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; 139:785.
  15. DeBaun MR, Gordon M, McKinstry RC, et al. Controlled trial of transfusions for silent cerebral infarcts in sickle cell anemia. N Engl J Med 2014; 371:699.
  16. Alvarez O, Yovetich NA, Scott JP, 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; 88:932.
  17. 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; 381:930.
  18. Vichinsky EP, Haberkern CM, Neumayr L, et al. A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease. The Preoperative Transfusion in Sickle Cell Disease Study Group. N Engl J Med 1995; 333:206.
  19. Vichinsky EP, Neumayr LD, Haberkern C, et al. The perioperative complication rate of orthopedic surgery in sickle cell disease: report of the National Sickle Cell Surgery Study Group. Am J Hematol 1999; 62:129.
  20. Waldron P, Pegelow C, Neumayr L, et al. Tonsillectomy, adenoidectomy, and myringotomy in sickle cell disease: perioperative morbidity. Preoperative Transfusion in Sickle Cell Disease Study Group. J Pediatr Hematol Oncol 1999; 21:129.
  21. Haberkern CM, Neumayr LD, Orringer EP, et al. Cholecystectomy in sickle cell anemia patients: perioperative outcome of 364 cases from the National Preoperative Transfusion Study. Preoperative Transfusion in Sickle Cell Disease Study Group. Blood 1997; 89:1533.
  22. Neumayr L, Koshy M, Haberkern C, et al. Surgery in patients with hemoglobin SC disease. Preoperative Transfusion in Sickle Cell Disease Study Group. Am J Hematol 1998; 57:101.
  23. Klings ES, Machado RF, Barst RJ, et al. 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; 189:727.
  24. Gladwin MT, Vichinsky E. Pulmonary complications of sickle cell disease. N Engl J Med 2008; 359:2254.
  25. Beverung LM, Strouse JJ, Hulbert ML, et al. Health-related quality of life in children with sickle cell anemia: impact of blood transfusion therapy. Am J Hematol 2015; 90:139.
  26. Sins JW, Biemond BJ, van den Bersselaar SM, et al. Early occurrence of red blood cell alloimmunization in patients with sickle cell disease. Am J Hematol 2016; 91:763.
  27. Vichinsky EP, Earles A, Johnson RA, et al. Alloimmunization in sickle cell anemia and transfusion of racially unmatched blood. N Engl J Med 1990; 322:1617.
  28. Ambruso DR, Githens JH, Alcorn R, et al. Experience with donors matched for minor blood group antigens in patients with sickle cell anemia who are receiving chronic transfusion therapy. Transfusion 1987; 27:94.
  29. Tahhan HR, Holbrook CT, Braddy LR, et al. Antigen-matched donor blood in the transfusion management of patients with sickle cell disease. Transfusion 1994; 34:562.
  30. Castilho L, Rios M, Bianco C, et al. DNA-based typing of blood groups for the management of multiply-transfused sickle cell disease patients. Transfusion 2002; 42:232.
  31. Castro O, Sandler SG, Houston-Yu P, Rana S. Predicting the effect of transfusing only phenotype-matched RBCs to patients with sickle cell disease: theoretical and practical implications. Transfusion 2002; 42:684.
  32. Afenyi-Annan A, Willis MS, Konrad TR, Lottenberg R. Blood bank management of sickle cell patients at comprehensive sickle cell centers. Transfusion 2007; 47:2089.
  33. Ameen R, Al Shemmari S, Al-Bashir A. Red blood cell alloimmunization among sickle cell Kuwaiti Arab patients who received red blood cell transfusion. Transfusion 2009; 49:1649.
  34. Vichinsky EP. The prevention and management of alloimmunization in sickle cell disease: the benefit of extended phenotypic matching of red blood cells. Immunohematology 2012; 28:20.
  35. Vichinsky EP, Luban NL, Wright E, et al. Prospective RBC phenotype matching in a stroke-prevention trial in sickle cell anemia: a multicenter transfusion trial. Transfusion 2001; 41:1086.
  36. Lasalle-Williams M, Nuss R, Le T, et al. Extended red blood cell antigen matching for transfusions in sickle cell disease: a review of a 14-year experience from a single center (CME). Transfusion 2011; 51:1732.
  37. Chou ST, Jackson T, Vege S, et al. High prevalence of red blood cell alloimmunization in sickle cell disease despite transfusion from Rh-matched minority donors. Blood 2013; 122:1062.
  38. Olujohungbe A, Hambleton I, Stephens L, et al. Red cell antibodies in patients with homozygous sickle cell disease: a comparison of patients in Jamaica and the United Kingdom. Br J Haematol 2001; 113:661.
  39. Aygun B, Padmanabhan S, Paley C, Chandrasekaran V. Clinical significance of RBC alloantibodies and autoantibodies in sickle cell patients who received transfusions. Transfusion 2002; 42:37.
  40. Price CL, Boyd JH, Watkins AR, et al. Mailing of a sickle cell disease educational packet increases blood donors within an African American community. Transfusion 2006; 46:1388.
  41. Osby M, Shulman IA. Phenotype matching of donor red blood cell units for nonalloimmunized sickle cell disease patients: a survey of 1182 North American laboratories. Arch Pathol Lab Med 2005; 129:190.
  42. Ribeiro KR, Guarnieri MH, da Costa DC, et al. DNA array analysis for red blood cell antigens facilitates the transfusion support with antigen-matched blood in patients with sickle cell disease. Vox Sang 2009; 97:147.
  43. Fichou Y, Mariez M, Le Maréchal C, Férec C. The experience of extended blood group genotyping by next-generation sequencing (NGS): investigation of patients with sickle-cell disease. Vox Sang 2016; 111:418.
  44. Fasano RM, Chou ST. Red Blood Cell Antigen Genotyping for Sickle Cell Disease, Thalassemia, and Other Transfusion Complications. Transfus Med Rev 2016; 30:197.
  45. da Costa DC, Pellegrino J Jr, Guelsin GA, et al. Molecular matching of red blood cells is superior to serological matching in sickle cell disease patients. Rev Bras Hematol Hemoter 2013; 35:35.
  46. Chadebech P, de Ménorval MA, Bodivit G, et al. Evidence of benefits from using fresh and cryopreserved blood to transfuse patients with acute sickle cell disease. Transfusion 2016; 56:1730.
  47. Desai PC, Deal AM, Pfaff ER, et al. Alloimmunization is associated with older age of transfused red blood cells in sickle cell disease. Am J Hematol 2015; 90:691.
  48. Fields ME, Hulbert ML, Chen L, et al. Red blood cell storage duration is not associated with clinical outcomes for acute chest syndrome in children with sickle cell disease. Transfusion 2015; 55:2714.
  49. Karafin MS, Singavi AK, Irani MS, et al. Red cell storage age policy for patients with sickle cell disease: A survey of transfusion service directors in the United States. Transfus Apher Sci 2016; 54:158.
  50. Cho CH, Yun SG, Koh YE, Lim CS. Effect of Irradiation on Microparticles in Red Blood Cell Concentrates. Ann Lab Med 2016; 36:362.
  51. Katharia R, Chaudhary R, Agarwal P. Prestorage gamma irradiation induces oxidative injury to red cells. Transfus Apher Sci 2013; 48:39.
  52. Ellison AM, Ota KV, McGowan KL, Smith-Whitley K. Epidemiology of bloodstream infections in children with sickle cell disease. Pediatr Infect Dis J 2013; 32:560.
  53. Naik RP, Streiff MB, Lanzkron S. Sickle cell disease and venous thromboembolism: what the anticoagulation expert needs to know. J Thromb Thrombolysis 2013; 35:352.
  54. Shah N, Landi D, Shah R, et al. Complications of implantable venous access devices in patients with sickle cell disease. Am J Hematol 2012; 87:224.
  55. Jeng MR, Feusner J, Skibola C, Vichinsky E. Central venous catheter complications in sickle cell disease. Am J Hematol 2002; 69:103.
  56. Zarrouk V, Habibi A, Zahar JR, et al. Bloodstream infection in adults with sickle cell disease: association with venous catheters, Staphylococcus aureus, and bone-joint infections. Medicine (Baltimore) 2006; 85:43.
  57. Schmalzer EA, Lee JO, Brown AK, et al. Viscosity of mixtures of sickle and normal red cells at varying hematocrit levels. Implications for transfusion. Transfusion 1987; 27:228.
  58. Hulbert ML, Scothorn DJ, Panepinto JA, et al. Exchange blood transfusion compared with simple transfusion for first overt stroke is associated with a lower risk of subsequent stroke: a retrospective cohort study of 137 children with sickle cell anemia. J Pediatr 2006; 149:710.
  59. Wahl SK, Garcia A, Hagar W, et al. Lower alloimmunization rates in pediatric sickle cell patients on chronic erythrocytapheresis compared to chronic simple transfusions. Transfusion 2012; 52:2671.
  60. Koehl B, Sommet J, Holvoet L, et al. Comparison of automated erythrocytapheresis versus manual exchange transfusion to treat cerebral macrovasculopathy in sickle cell anemia. Transfusion 2016; 56:1121.
  61. Kim HC, Dugan NP, Silber JH, et al. Erythrocytapheresis therapy to reduce iron overload in chronically transfused patients with sickle cell disease. Blood 1994; 83:1136.
  62. Hilliard LM, Williams BF, Lounsbury AE, Howard TH. Erythrocytapheresis limits iron accumulation in chronically transfused sickle cell patients. Am J Hematol 1998; 59:28.
  63. Singer ST, Quirolo K, Nishi K, et al. Erythrocytapheresis for chronically transfused children with sickle cell disease: an effective method for maintaining a low hemoglobin S level and reducing iron overload. J Clin Apher 1999; 14:122.
  64. Savage WJ, Reddoch S, Wolfe J, Casella JF. Partial manual exchange reduces iron accumulation during chronic red cell transfusions for sickle cell disease. J Pediatr Hematol Oncol 2013; 35:434.
  65. Charache S. Treatment of sickle cell anemia. Annu Rev Med 1981; 32:195.
  66. Carrara P, Balocco M, Pinto V, et al. Manual erythroexchange for chronic transfusion therapy in patients with sickle cell syndromes unresponsive to hydroxyurea: a long-term follow-up. Am J Hematol 2010; 85:974.
  67. Nieburg PI, Stockman JA. Rapid correction of anemia with partial exchange transfusion. Am J Dis Child 1977; 131:60.
  68. Casella JF, King AA, Barton B, et al. Design of the silent cerebral infarct transfusion (SIT) trial. Pediatr Hematol Oncol 2010; 27:69.
  69. Ballas SK. Meperidine for acute sickle cell pain in the emergency department: revisited controversy. Ann Emerg Med 2008; 51:217.
  70. Morgan MT. Use of meperidine as the analgesic of choice in treating pain from acute painful sickle cell crisis. Ann Emerg Med 2008; 51:202.
  71. Rosse WF, Gallagher D, Kinney TR, et al. Transfusion and alloimmunization in sickle cell disease. The Cooperative Study of Sickle Cell Disease. Blood 1990; 76:1431.
  72. Yazdanbakhsh K, Ware RE, Noizat-Pirenne F. Red blood cell alloimmunization in sickle cell disease: pathophysiology, risk factors, and transfusion management. Blood 2012; 120:528.
  73. de Montalembert M, Dumont MD, Heilbronner C, et al. Delayed hemolytic transfusion reaction in children with sickle cell disease. Haematologica 2011; 96:801.
  74. Chadebech P, Habibi A, Nzouakou R, et al. Delayed hemolytic transfusion reaction in sickle cell disease patients: evidence of an emerging syndrome with suicidal red blood cell death. Transfusion 2009; 49:1785.
  75. Garratty G. Severe reactions associated with transfusion of patients with sickle cell disease. Transfusion 1997; 37:357.
  76. Petz LD, Calhoun L, Shulman IA, et al. The sickle cell hemolytic transfusion reaction syndrome. Transfusion 1997; 37:382.
  77. Vidler JB, Gardner K, Amenyah K, et al. Delayed haemolytic transfusion reaction in adults with sickle cell disease: a 5-year experience. Br J Haematol 2015; 169:746.
  78. Dumas G, Habibi A, Onimus T, et al. Eculizumab salvage therapy for delayed hemolysis transfusion reaction in sickle cell disease patients. Blood 2016; 127:1062.
  79. Bachmeyer C, Maury J, Parrot A, et al. Rituximab as an effective treatment of hyperhemolysis syndrome in sickle cell anemia. Am J Hematol 2010; 85:91.
  80. Herwaldt BL, Linden JV, Bosserman E, et al. Transfusion-associated babesiosis in the United States: a description of cases. Ann Intern Med 2011; 155:509.
  81. Mace KE, Arguin PM. Malaria Surveillance - United States, 2014. MMWR Surveill Summ 2017; 66:1.
  82. Ballas SK. Iron overload is a determinant of morbidity and mortality in adult patients with sickle cell disease. Semin Hematol 2001; 38:30.
  83. 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; 115:2354.
  84. Harmatz P, Butensky E, Quirolo K, et al. Severity of iron overload in patients with sickle cell disease receiving chronic red blood cell transfusion therapy. Blood 2000; 96:76.
  85. Darbari DS, Kple-Faget P, Kwagyan J, et al. Circumstances of death in adult sickle cell disease patients. Am J Hematol 2006; 81:858.
  86. Olivieri NF. Progression of iron overload in sickle cell disease. Semin Hematol 2001; 38:57.
  87. Perronne V, Roberts-Harewood M, Bachir D, et al. Patterns of mortality in sickle cell disease in adults in France and England. Hematol J 2002; 3:56.
  88. Porter JB, Shah FT. Iron overload in thalassemia and related conditions: therapeutic goals and assessment of response to chelation therapies. Hematol Oncol Clin North Am 2010; 24:1109.
  89. Brittenham GM. Iron-chelating therapy for transfusional iron overload. N Engl J Med 2011; 364:146.
  90. Lucania G, Vitrano A, Filosa A, Maggio A. Chelation treatment in sickle-cell-anaemia: much ado about nothing? Br J Haematol 2011; 154:545.
  91. Berdoukas V, Nord A, Carson S, et al. Tissue iron evaluation in chronically transfused children shows significant levels of iron loading at a very young age. Am J Hematol 2013; 88:E283.
  92. Berdoukas V, Farmaki K, Wood JC, Coates T. Iron chelation in thalassemia: time to reconsider our comfort zones. Expert Rev Hematol 2011; 4:17.
  93. Brittenham GM, Cohen AR, McLaren CE, et al. Hepatic iron stores and plasma ferritin concentration in patients with sickle cell anemia and thalassemia major. Am J Hematol 1993; 42:81.
  94. Pippard MJ. Iron overload and iron chelation therapy in thalassaemia and sickle cell haemoglobinopathies. Acta Haematol 1987; 78:206.
  95. Karam LB, Disco D, Jackson SM, et al. Liver biopsy results in patients with sickle cell disease on chronic transfusions: poor correlation with ferritin levels. Pediatr Blood Cancer 2008; 50:62.
  96. Villeneuve JP, Bilodeau M, Lepage R, et al. Variability in hepatic iron concentration measurement from needle-biopsy specimens. J Hepatol 1996; 25:172.
  97. Meloni A, Puliyel M, Pepe A, et al. Cardiac iron overload in sickle-cell disease. Am J Hematol 2014; 89:678.
  98. Walter PB, Harmatz P, Vichinsky E. Iron metabolism and iron chelation in sickle cell disease. Acta Haematol 2009; 122:174.
  99. Walter PB, Fung EB, Killilea DW, et al. Oxidative stress and inflammation in iron-overloaded patients with beta-thalassaemia or sickle cell disease. Br J Haematol 2006; 135:254.
  100. Porter J, Garbowski M. Consequences and management of iron overload in sickle cell disease. Hematology Am Soc Hematol Educ Program 2013; 2013:447.
  101. Wang CJ, Kavanagh PL, Little AA, et al. Quality-of-Care Indicators for Children With Sickle Cell Disease. Pediatrics 2011; 128:484.
  102. Coates TD, Carson S, Wood JC, Berdoukas V. Management of iron overload in hemoglobinopathies: what is the appropriate target iron level? Ann N Y Acad Sci 2016; 1368:95.
  103. Vichinsky E, Onyekwere O, Porter J, et al. A randomised comparison of deferasirox versus deferoxamine for the treatment of transfusional iron overload in sickle cell disease. Br J Haematol 2007; 136:501.
  104. Cohen AR, Galanello R, Piga A, et al. Safety and effectiveness of long-term therapy with the oral iron chelator deferiprone. Blood 2003; 102:1583.
  105. Vichinsky E, Torres M, Minniti CP, et al. Efficacy and safety of deferasirox compared with deferoxamine in sickle cell disease: two-year results including pharmacokinetics and concomitant hydroxyurea. Am J Hematol 2013; 88:1068.
  106. http://www.accessdata.fda.gov/drugsatfda_docs/label/2009/021882s006lbl.pdf (Accessed on October 27, 2015).
  107. http://www.accessdata.fda.gov/drugsatfda_docs/label/2015/206910s001s003lbl.pdf (Accessed on October 27, 2015).
  108. Vichinsky E, Bernaudin F, Forni GL, et al. Long-term safety and efficacy of deferasirox (Exjade) for up to 5 years in transfusional iron-overloaded patients with sickle cell disease. Br J Haematol 2011; 154:387.
  109. http://www.accessdata.fda.gov/drugsatfda_docs/label/2015/021882s021lbl.pdf (Accessed on August 03, 2015).
  110. Baum M. Renal Fanconi syndrome secondary to deferasirox: where there is smoke there is fire. J Pediatr Hematol Oncol 2010; 32:525.
  111. Dubourg L, Laurain C, Ranchin B, et al. Deferasirox-induced renal impairment in children: an increasing concern for pediatricians. Pediatr Nephrol 2012; 27:2115.
  112. Grangé S, Bertrand DM, Guerrot D, et al. Acute renal failure and Fanconi syndrome due to deferasirox. Nephrol Dial Transplant 2010; 25:2376.
  113. Wong P, Polkinghorne K, Kerr PG, et al. Deferasirox at therapeutic doses is associated with dose-dependent hypercalciuria. Bone 2016; 85:55.
  114. Vichinsky E. Iron overload and iron chelation therapy in pediatric patients. US Hematology 2009; 2:64. Online access: http://www.touchbriefings.com/pdf/3324/elliott_vichinsky.pdf
  115. Kwiatkowski JL. Management of transfusional iron overload - differential properties and efficacy of iron chelating agents. J Blood Med 2011; 2:135.
  116. Lal A, Sweeters N, Ng V, et al. Combined chelation therapy with deferasirox and deferoxamine in transfusion-dependent thalassemia. Blood (ASH Annual Meeting Abstracts) 2010; 116:4269.
  117. Davis BA, Porter JB. Long-term outcome of continuous 24-hour deferoxamine infusion via indwelling intravenous catheters in high-risk beta-thalassemia. Blood 2000; 95:1229.
  118. Pennell DJ, Udelson JE, Arai AE, et al. Cardiovascular function and treatment in β-thalassemia major: a consensus statement from the American Heart Association. Circulation 2013; 128:281.
  119. 2012 Standards of Care Guidelines for Thalassemia. Published by Children's Hospital & Research Center Oakland. http://hemonc.cho.org/thalassemia/treatment-guidelines-1.aspx#gsc.tab=0 and http://hemonc.cho.org/thalassemia/documents/SOCGuidelines2012.pdf (Accessed on October 08, 2013).
  120. Olivieri NF, Buncic JR, Chew E, et al. Visual and auditory neurotoxicity in patients receiving subcutaneous deferoxamine infusions. N Engl J Med 1986; 314:869.
  121. http://www.pharma.us.novartis.com/product/pi/pdf/exjade.pdf (Accessed on March 07, 2014).
  122. Fung EB, Harmatz PR, Lee PD, et al. Increased prevalence of iron-overload associated endocrinopathy in thalassaemia versus sickle-cell disease. Br J Haematol 2006; 135:574.