TRANSFUSION PRACTICE, POST-TRANSFUSION COMPLICATIONS AND RISK FACTORS IN SICKLE CELL DISEASE IN SENEGAL, WEST AFRICA.
Main Article Content
Blood transfusion, Sickle cell disease, Infectious, Alloimmunization, Iron overload
Context and Objectives: Blood transfusions (BT) remain a mainstay of therapy for patients with sickle cell disease (SCD), but pose significant clinical challenges. We aim to assess infectious markers, red cell alloimmunization and iron overload secondary to BT in SCD patients.
Materials and Methods: This is a case-control study included 253 SCD (153 SCD-transfused and 100 SCD non-transfused). We evaluated the transfusion practice (modalities, indications), post-transfusion complications (infections, alloimmunization, iron overload) and risk factors of these complications (socio-demographic, clinical, biological).
Results: Median age was 28.5 years (5 - 59). Sex ratio was 0.86. Homozygous SCD was more common (95.3%). Simple BT was performed in 92.8% and transfusion exchange in 18.9%. Transfusion indications were dominated by acute anemia (57.06%) and vaso-occlusive crisis (VOCs) (14%). Red blood cell concentrates (RBC) were administered to 93.46%. Median number of RBC received per patient was 10 (2 - 48). The prevalence of VHC in SCD-transfused was 1.33% and 2% for VHB. Anti-HIV antibodies were not found. Red cell alloimmunization frequency was 16%. The most common alloantibodies were anti-rhesus (34.19%) and anti-Kell (23.67%). Iron overload was detected in 7.84%. The number of RBC transfused was the only risk factor for alloimmunization (p = 0.03) and iron overload (p = 0.023). BT frequency was not related to infectious transmission.
Conclusion: Despite advances in blood safety, BT therapy is still a risk for SCD polytransfused patients. Although infectious transmission has rare, the risk of alloimmunization and iron overload is high in these patients.
2. Samir KB. Sickle cell disease: Classification of clinical complications and approaches to preventive and therapeutic management. Clin Hemorheol Microcirc. 2018;68(2-3):105-128
3. Stella TC, Ross MF. Management of Patients with Sickle Cell Disease Using Transfusion Therapy. Guidelines and Complications. Hematol Oncol Clin N Am 30 (2016) 591–608.
4. Stella TC, Mouaz A, Ross MF, Joshua JF, Jeanne EH, Jo H, Kameka M, Kwiatkowski JL, Pirenne F, Shi PA, Stowell SR, Thein SL, Westhoff CM, Wong TE, Akl EA. American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support. Blood Advances 2019:4(2): 327-355. Blood Adv. 2020 Jan 28;4(2):327-355
5. Sharma D, Ogbenna AA, Kassim A, Andrews J. Transfusion support in patients with sickle cell disease. Semin Hematol. 2020 Apr;57(2):39-50
6. Faye BF, Sow D, Seck M, Dieng N, Touré SA, Gadji M, Senghor AB, Gueye YB, Sy D, Sall A, Dieye TN, Toure AO, Diop S . Efficacy and Safety of Manual Partial Red Cell Exchange in the Management of Severe Complications of Sickle Cell Disease in a Developing Country. Adv Hematol. 2017;2017:3518402. doi: 10.1155/2017/3518402. Epub 2017 May 11.
7. Mabien AP, Brown B, Herbert DE, Haynes J. Iron overload in adults with sickle cell disease who have received intermittent red blood cell transfusions. J Am Assoc Nurse Pract. 2015; 27(10):591-6.
8. Pirenne F. The cause and pathogenesis of hemolytic transfusion reactions in sickle-cell disease. Curr Opin Hematol. 2019 Nov;26(6):488-494.
9. Grace EL and Stella TC. Red cell transfusion and alloimmunization in sickle cell disease. Haematologica. 2021 Jul 1;106(7):1805-1815
10. Allali S, Peyrard T, Amiranoff D, Jérémie FC, Chalumeau M, Brousse V, Montalembert M. Prevalence and risk factors for red blood cell alloimmunization in 175 children with sickle cell disease in a French university hospital reference centre. Br J Haematol.2017 May;177(4):641-647.
11. Seck M, Diéye B, Guéye YB, Faye BF, Senghor AB, Toure SA, Dieng N, Sall A, Touré AO, Diéye TN, Diop S. Evaluation of the efficacy of medical screening of blood donors on preventing blood transfusion-transmitted infectious agents. Transfus Clin Biol. 2016;23(2):98-102
12. Seck M, Tall A, Faye BF, Bah DS, Guéye Y, Sall A, Touré AO, Diop S. Evaluation of transfusion practices in sickle cell disease in Senegal: cohort study of 1078 patients with sickle cell disease. Med Sante Trop. 2017 Nov 1;27(4):402-406
13. Thomas DC, John CW. How we manage iron overload in sickle cell patients. Br J Haematol.2020. doi: 10.1111/bjh.14575
14. Blatyta PF, Kelly S, Sabino E, Preiss L, Mendes F, Carneiro-Proietti AB. Prevalence of serological markers of transfusion and sexually transmitted infections and their correlation with clinical features in a large cohort of Brazilian sickle cell disease patients. Transfusion. 2020; 60(2): 343–350.
15. Sally ACL, Kristina G, Mia CK, Yi-Fan C, Santosh LS, Lewis LH, Gordeuk VR, Ronald G Strauss RG, Triulzi DJ. Red blood cell alloimmunization in sickle cell disease: assessment of transfusion protocols during two time periods. Transfusion. 2018 Jul;58(7):1588-1596
16. Boulat C. La transfusion du drépanocytaire. Transfus. Clin. Biol. 2013 ; 20 :68-71
17. Elira Dokekias A, Ngolet Ossini L, Atipo Tsiba FO. Blood transfusion assessment to 112 homozygous sickle-cell disease patients in university hospital of Brazzaville.
Transfus Clin Biol. Nov-Dec 2009;16(5-6):464-70.
18. Krystalyn EH, Ross MF, Matthew SK, Jeanne EH, Richard OF. Mechanisms of alloimmunization in sickle cell disease. Curr Opin Hematol 2019, 26:434–441
19. Ross MF, Erin KM, Jane B, Mia SW, Robert WG, James RE. Impact of Red Blood Cell Antigen Matching on Alloimmunization and Transfusion Complications in Patients with Sickle Cell Disease: A Systematic Review. Ytmrv (2018), doi:10.1016/j.tmrv.2018.07.003.
20. Ben AI, Louati N, Khemekhem H, Dhieb A, Rekik H, Mdhaffar M, Gargouri J. Red blood cell immunization in haemoglobinopathie: about 84 cases. Transfus Clin Biol. 2012 Dec;19(6):345-52
21. Zalpuri S, Zwaginga JJ, le Cessie S, Elshuis J, Schonewille H, Van der bom JG. Red-blood-cell allo-immunization and number of red-blood-cell transfusions. Vox Sang. 2012 Feb;102(2):144-9.
22. Balbuena-Merle R, Hendrickson JE. Red blood cell alloimmunization and delayed hemolytic transfusion reactions in patients with sickle cell disease. Transfus Clin Biol, 2019 ;26 : 112–115
23. Noizat-Pirenne F. Immunohematologic characteristics in the Afro-caribbean population. Consequences for transfusion safety. Transfus Clin Biol. 2003 Jun;10(3):185-91.
24. Kangiwa U, Ibegbulam O, Ocheni S, Madu A, Mohammed N. Pattern and prevelence of alloimmunization in multiply transfused patients with sickle cell disease in Nigeria. Biomark Res. 2015 Oct 13; 3: 26.
25. Dias Zanette AM, de Souza Goncalves M, Vilasboas Schettini L, Magalhaes Agguiar L, Santos Bahia RC, Vasconcelos Nogueira LA, de Freitas Brandão CJ, Neves de Azevedo AC, Ramos de Aragao L, Marcos Arruda S. Alloimmunisation and clinical proﬁle of sickle cell disease patients from Salvador-Brazil. Ethn Dis. Spring 2010;20(2):136-41.
26. Aygun B, Padmanabhan S, Paley C, Chandrasekaran V. Clinical significance of RBC alloantibodies and autoantibodies in sickle cell patients who received transfusions. Transfusion 2002; 42:37-43.
27. Higgins JM, Sloan SR. Stochastic modeling of human RBC alloimmunization: evidence for a distinct population of immunologic responders. Blood. 2008; 112:2546-53.
28. Lilian AB, Andrew DC, Robertson DD, Alex OA, Sheri HA, Henk S. Red blood cell alloimmunization and minor red blood cell antigen phenotypes in transfused Ghanaian patients with sickle cell disease. Transfusion. 2019;9999;1–7
29. Boateng LA, Ngoma AM, Bates I, Schonewille H. Red Blood Cell Alloimmunization in Transfused Patients With Sickle Cell Disease in Sub-Saharan Africa; a Systematic Review and Meta-Analysis. Transfus Med Rev. 2019 Jul;33(3):162-169
30. Stanley HM, Friedman DF, Webb J, Kwiatkowski JL. Transfusional Iron Overload in a Cohort of Children with Sickle Cell Disease: Impact of Magnetic Resonance Imaging, Transfusion Method, and Chelation. Pediatr Blood Cancer 2016;63:1414–1418
31. Oduor H, Minniti CP, Brofferio A, Gharib AM, Abd-Elmoniem KZ, Hsieh MM, Tisdale JF, Fitzhugh CD. Severe cardiac iron toxicity in two adults with sickle cell disease. Transfusion. 2017 Mar;57(3):700-704.
32. Hafsia R, Belakhal F, Ben Salah N, Gouider E, Elborji W. Iron overload in sickle cell anemia : a study of 94 patients. Tunis Med. 2011 Jun;89(6):548-52.
33. Leo-Kodeli S, Renaudier P, Lassale B. Evaluation of transfusion hemochromatosis prevalence, SFVTT-01 study: preliminary results of the SFVTT working group. Transfus Clin Biol. 2014 Nov;21(4-5):182-8.
34. Ginwalla M, AlMasoud A, Tofovic D, Alin T, Al-Kindi S, Oliveira G, Rajagopalan S, Schilz R, Little J. Cardiovascular Evaluation and Management of Iron Overload Cardiomyopathy in Sickle Cell Disease. Am J Hematol. 2018 Jan;93(1):E7-E9. doi: 10.1002/ajh.24924. Epub 2017 Oct 23.
35. Yassin M, Soliman A, De Sanctis V, Nashwan A, Abusamaan S, Moustafa A, Samah K, Soliman D. Liver iron content (LIC) in adults with sickle cell disease (SCD): correlation with serum ferritin and liver enzymes concentrations in trasfusion dependent (TD-SCD) and non-transfusion dependent (NT-SCD) patients. Mediterr J Hematol Infect Dis. 2017 Jun 20;9(1):e2017037. doi: 10.4084/MJHID.2017.037. eCollection 2017
36. Thuret I, Barlogis V, Michel G. Current concepts in the management of transfusional iron overload. Arch Pediatr. 2009 Jun;16(6):559-61.
37. Walter PB, Fung EB, Killilea DW, Jiang Q, Hudes M, Madden J, Porter J, Patricia E, Vichinsky E, Harmatz P. Oxidative stress and inflammation in iron-overloaded patients with beta-thalassaemia or sickle cell disease. Br J Haematol. 2006 Oct;135(2):254-63.
38. Akinbami AA, Dosunmu AO, Adediran AA, Oshinaike OO, Osunkalu VO, Ajibola SO, Arogundade OM. Serum ferritin levels in adults with sickle cell disease in Lagos, Nigeria. J Blood Med. 2013 May 22;4:59-63
39. Gehrie EA, Ness PM, Bloch EM, Kacker S, Tobian AA. Medical and economic implications of strategies to prevent alloimmunization in sickle cell disease. Transfusion. 2017 Sep;57(9):2267-2276.
40. Meinderts SM, Gerritsma JJ, Sins JW, de Boer M, van Leeuwen K, Biemond BJ, Rijneveld AW, Kerkhoffs Jean-Louis H, Habibi A, Bruggen RV, Kuijpers TW, Schoot EVD, Pirenne F, Fijnvandraat K, Tanck MW, Van den Berg TK. Identification of genetic biomarkers for alloimmunization in sickle cell disease. Br J Haematol. 2019 Sep;186(6):887-899.
41. Noizat-Pirenne F. Transfusion and sickle cell disease: axes of transfusion safety optimization. Transfus Clin Biol. 2014 May;21(2):77-84.
42. Dedeken L, Quoc LP, Rozen L, El Kenz H, Huybrechts S, Devalck C, Diallo S, Heijmans C, Ferster A. Automated RBC exchange compared to manual exchange transfusion for children with sickle cell disease is cost-effective and reduces iron overload. Transfusion. 2018 Jun;58(6):1356-1362.
43. Diarra AB, Guindo A, Kouriba B, Dorie A. Sickle cell anemia and transfusion safety in Bamako, Mali. Seroprevalence of HIV, HBV and HCV infections and alloimmunization belonged to Rh and Kell systems in sickle cell anemia patients. Transfus Clin Biol. 2013 Dec;20(5-6):476-81.
44. Ngo-Sack F, Noah D, Zouhaïratou H, Mbanya D. Prevalence of HBsAg and anti-HCV antibodies in homozygous sickle cell patients at Yaounde Central Hospital. Pan Afr Med J. 2013;14:40.doi: 10.11604/pamj.2013.14.40.2069. Epub 2013 Jan 28.
45. Agence National de la Statistique et de la Démographie (ANSD): Rapport sur la prévalence des infections au Sénégal en 2016. https://www.sec.gouv.sn/agence-nationale
46. Kissou SA, Koura M, Sawadogo A, Ouédraogo AS, Traoré H, Kamboulé K, Zogona WWF, Nacro B. Serological Markers of Viral Hepatitis B and C in Children with Sickle Cell Disease Monitored in the Pediatrics Department at the University Hospital of Bobo-Dioulasso (Burkina Faso). Bull Soc Pathol Exot. 2017 Aug;110(3):160-164.
47. Fasola FA, Odaibo GN, Aken’Ova YA, Olaleye OD. Hepatitis B and C viral markers in patients with sickle cell disease in Ibadan, Nigeria. Afr J Med Sci, 2003; 32: 293 -295.
48. Séka-séka J, Yapo-Crezoit AC, Dasse-Sery R, Akre-Draga P, Sorho F, Sombo Mambo F. Etude de la séroprévalence de l’hépatite virale C dans la population drépanocytaire en Côte d’Ivoire. Méd Afr N, 1998; 45 (1):102-10
49. Schreiber GB, Busch MP, Kleinman SH, Korelitz JJ. The risk of transfusion-transmitted viral infections. The Retrovirus Epidemiology Donor Study. N Engl J Med. 1996 Jun 27;334(26):1685-90.
50. Uwingabiye J, Zahid H, Unyendie L, Hadef R. Seroprevalence of viral markers among blood donors at the Blood Donor Center of Mohammed V Military Teaching Hospital of Rabat, Morocco. Pan Afr Med J. 2016 Nov 24;25:185.
51. Karafina MS, Carpenterb E, Panb A, Simpsonb P, Field JJ. Older red cell units are associated with an increased incidence ofinfection in chronically transfused adults with sickle cell disease. Transfus Apher Sci. 2017 Jun;56(3):345-351
52. Touré-Fall AO, Dièye TND, Sall A, Diop M, Diop S, Thiam D, Diakhate L. Residual risk of transmission of HIV and HBV, in Senegalese national blood bank from 2003 to 2005. Transfus Clin Biol. Nov-Dec 2009;16(5-6):439-43.