Stroke Management in Sickle Cell Disease Patients in Basra: A Single-Centre on Erythrocytapheresis as a Secondary Prevention Strategy
Main Article Content
Abstract
Background: Sickle cell disease is a major hereditary haemoglobinopathy in Basrah;Iraq. Stroke is one of the most devastating complications of Sickle cell disease and chronic erythrocytapheresis has become a cornerstone of its secondary prevention.
Objectives: To describe the clinical profile, complication burden and outcomes of Stroke in Sickle cell disease patients attending the Basrah Center for Hereditary Blood Diseases and to evaluate the safety and efficacy of erythrocytapheresis as a secondary Stroke-prevention strategy.
Methods: A descriptive study was conducted at Basrah Center for Hereditary Blood Diseases. Sixteen patients with confirmed Sickle cell disease and stroke who maintained on a regular erythrocytapheresis program.
Results: Twelve patients (75.0%) were male and four (25.0%) were female; ages ranged from 8 to 23 years (mean 15.1 years). A single prior Stroke was documented in 50% patients, two episodes in 18.75% and more than two episodes in 31.25%. Stroke recurrence showed 50.0% of children aged ≤10 years had ≥3 prior events.Cumulative transfusion exposure was variable (< 10 simple transfusions in 4/16, 10–30 in 5/16 and qualitatively reported as “frequent” in 6/16), and 4 of 16 patients had more than 25 cumulative erythrocytapheresis sessions. Service attitudes were 93.75% strongly agreed that the apheresis service is indispensable to the center, 93.75% were “very satisfied” with the service and 62.50% reported a marked improvement in life and function after enrolment.
Conclusions: Erythrocytopheresis combined with hydroxyurea provides effective and safe secondary Stroke prevention in Sickle cell disease patients at Basrah Center for Hereditary Blood Diseases, with a low recurrence rate (12.5%) and substantial improvements.
Keywords:
Sickle cell disease, Stroke, Erythrocytapheresis, Red cell exchange, Secondary prevention, Hydroxyurea, Basra, Iraq
Article Details
How to Cite
ALHIJAJ, Basim Abdulkareem et al.
Stroke Management in Sickle Cell Disease Patients in Basra: A Single-Centre on Erythrocytapheresis as a Secondary Prevention Strategy.
Medical Research Archives, [S.l.], v. 14, n. 7, july 2026.
ISSN 2375-1924.
Available at: <https://esmed.org/MRA/mra/article/view/7645>. Date accessed: 08 aug. 2026.
doi: https://doi.org/10.18103/mra.v14i7.7645.
Section
Research Articles
The Medical Research Archives grants authors the right to publish and reproduce the unrevised contribution in whole or in part at any time and in any form for any scholarly non-commercial purpose with the condition that all publications of the contribution include a full citation to the journal as published by the Medical Research Archives.
References
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10. Jumaa DS, Al Hijaj BA, Dixen JZ. Erythrocytapheresis service in Basrah: safety and efficacy statistics. Eur J Med Health Res. 2024;2(5):318-326. Accessed May 27, 2026. Available at: https://ejmhr.com/index.php/journal/article/view/316
11. Kassim AA, Galadanci NA, Pruthi S, DeBaun MR. How I treat and manage strokes in sickle cell disease. Blood. 2015;125(22):3401-3410. doi:10.1182/blood-2014-09-551564
12. Ohene-Frempong K, Weiner SJ, Sleeper LA, Miller ST, Embury SH, Moohr JW, et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood. 1998;91(1):288-294.
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16. Switzer JA, Hess DC, Nichols FT, Adams RJ. Pathophysiology and treatment of stroke in sickle-cell disease: present and future. Lancet Neurol. 2006;5(6):501-512. doi:10.1016/S1474-4422(06)70469-0
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19. Powars D, Wilson B, Imbus C, Pegelow C, Allen J. The natural history of stroke in sickle cell disease. Am J Med. 1978;65(3):461-471. doi:10.1016/0002-9343(78)90772-6
20. Balkaran B, Char G, Morris JS, Thomas PW, Serjeant BE, Serjeant GR. Stroke in a cohort of patients with homozygous sickle cell disease. J Pediatr. 1992;120(3):360-366. doi:10.1016/S0022-3476(05)80897-2
21. Adams RJ, McKie VC, Hsu L, Files B, Vichinsky E, Pegelow C, 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. doi:10.1056/NEJM199807023390102
22. Lee MT, Piomelli S, Granger S, Miller ST, Harkness S, Brambilla DJ, et al. Stroke Prevention Trial in Sickle Cell Anemia (STOP): extended follow-up and final results. Blood. 2006;108(3):847-852. doi:10.1182/blood-2005-10-009506
23. Bernaudin F, Verlhac S, Arnaud C, Kamdem A, Chevret S, Hau I, et al. Impact of early transcranial Doppler screening and intensive therapy on cerebral vasculopathy outcome in a newborn sickle cell anemia cohort. Blood. 2011;117(4):1130-1140. doi:10.1182/blood-2010-06-293514
24. Ware RE, Davis BR, Schultz WH, Brown RC, Aygun B, Sarnaik S, et al. Hydroxycarbamide versus chronic transfusion for maintenance of transcranial Doppler flow velocities in children with sickle cell anaemia—TCD With Transfusions Changing to Hydroxyurea (TWiTCH): a multicentre, open-label, phase 3, non-inferiority trial. Lancet. 2016;387(10019):661-670. doi:10.1016/S0140-6736(15)01041-7
25. Ware RE, Helms RW; SWiTCH Investigators. Stroke With Transfusions Changing to Hydroxyurea (SWiTCH). Blood. 2012;119(17):3925-3932. doi:10.1182/blood-2011-11-392340
26. Helton KJ, Adams RJ, Kesler KL, Lockhart A, Aygun B, Driscoll C, et al. Magnetic resonance imaging/angiography and transcranial Doppler velocities in sickle cell anemia: results from the SWiTCH trial. Blood. 2014;124(6):891-898. doi:10.1182/blood-2013-12-545186
27. Wang WC, Ware RE, Miller ST, Iyer RV, Casella JF, Minniti CP, et al. Hydroxycarbamide in very young children with sickle-cell anaemia: a multicentre, randomised, controlled trial (BABY HUG). Lancet. 2011;377(9778):1663-1672. doi:10.1016/S0140-6736(11)60355-3
28. Schwartz J, Padmanabhan A, Aqui N, Balogun RA, Connelly-Smith L, Delaney M, et al. Guidelines on the use of therapeutic apheresis in clinical practice: evidence-based approach from the Writing Committee of the American Society for Apheresis: the seventh special issue. J Clin Apher. 2016;31(3):149-162. doi:10.1002/jca.21470
29. Padmanabhan A, Connelly-Smith L, Aqui N, Balogun RA, Klingel R, Meyer E, et al. Guidelines on the use of therapeutic apheresis in clinical practice: evidence-based approach from the Writing Committee of the American Society for Apheresis: the eighth special issue. J Clin Apher. 2019;34(3):171-354. doi:10.1002/jca.21705.
30. Sarode R, Matevosyan K, Rogers ZR, Burner JD, Rutherford CJ. Advantages of isovolemic hemodilution-red cell exchange therapy to prevent recurrent stroke in sickle cell anemia patients. J Clin Apher. 2011;26(4):200-207. doi:10.1002/jca.21203
31. Adams DM, Schultz WH, Ware RE, Kinney TR. Erythrocytapheresis can reduce iron overload and prevent the need for chelation therapy in chronically transfused pediatric patients. J Pediatr Hematol Oncol. 1996;18(1):46-50. doi:10.1097/00043426-199602000-00009
32. Hilliard LM, Williams BF, Lounsbury AE, Howard TH. Erythrocytapheresis limits iron accumulation in chronically transfused sickle cell patients. Am J Hematol. 1998;59(1):28- 35. doi:10.1002/(SICI)1096-8652(199809)59:1<28: AID-AJH6>3.0.CO;2-1
33. Kim HC. Red cell exchange: special focus on sickle cell disease. Hematology Am Soc Hematol Educ Program. 2014;2014(1):450-456. doi:10.1182/asheducation-2014.1.450
34. Lawson SE, Oakley S, Smith NA, Bareford D. Red cell exchange in sickle cell disease. Clin Lab Haematol. 1999;21(2):99-102. doi:10.1046/j.1365-2257.1999.00192.x
35. Tsitsikas DA, Sirigireddy B, Nzouakou R, Calvey A, Quinn J, Collins J, et al. Safety, tolerability, and outcomes of regular automated red cell exchange transfusion in the management of sickle cell disease. J Clin Apher. 2016;31(6):545-550. doi:10.1002/jca.21447
36. Verduzco LA, Nathan DG. Sickle cell disease and stroke. Blood. 2009;114(25):5117-5125. doi:10.1182/blood-2009-05-220921.
37. Brousse V, Buffet P, Rees D. The spleen and sickle cell disease: the sick(led) spleen. Br J Haematol. 2014;166(2):165-176. doi:10.1111/bjh.12950
38. Chou ST, Alsawas M, Fasano RM, Field JJ, Hendrickson JE, Howard J, et al. American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support. Blood Adv. 2020;4(2):327-355. doi:10.1182/bloodadvances.2019001143
39. Charache S, Terrin ML, Moore RD, Dover GJ, Barton FB, Eckert SV, et al. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. N Engl J Med. 1995;332(20):1317-1322. doi:10.1056/NEJM199505183322001
40. Steinberg MH, Barton F, Castro O, Pegelow CH, Ballas SK, Kutlar A, 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;289(13):1645-1651. doi:10.1001/jama.289.13.1645
41. Voskaridou E, Christoulas D, Bilalis A, Plata E, Varvagiannis K, Stamatopoulos G, 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(12):2354-2363. doi:10.1182/blood-2009-05-221333
42. Strouse JJ, Lanzkron S, Beach MC, Haywood C, Park H, Witkop C, et al. Hydroxyurea for sickle cell disease: a systematic review for efficacy and toxicity in children. Pediatrics. 2008;122(6):1332-1342. doi:10.1542/peds.2008-0441
43. Aljuburi G, Laverty AA, Green SA, Phekoo KJ, Banarsee R, Okoye NV, et al. Trends in hospital admissions for sickle cell disease in England, 2001/02-2009/10. J Public Health (Oxf). 2012;34(4):570-576. doi:10.1093/pubmed/fds035
44. Galadanci N, Wudil BJ, Balogun TM, Ogunrinde GO, Akinsulie A, Hasan-Hanga F, et al. Current sickle cell disease management practices in Nigeria. Int Health. 2014;6(1):23-28. doi:10.1093/inthealth/iht022
45. Panepinto JA, Bonner M. Health-related quality of life in sickle cell disease: past, present, and future. Pediatr Blood Cancer. 2012;59(2):377-385. doi:10.1002/pbc.24176
46. Schatz J, Brown RT, Pascual JM, Hsu L, DeBaun MR. Poor school and cognitive functioning with silent cerebral infarcts and sickle cell disease. Neurology. 2001;56(8):1109-11. doi:10.1212/wnl.56.8.1109. PMID: 11320190.
2. Piel FB, Steinberg MH, Rees DC. Sickle cell disease. N Engl J Med. 2017;376(16):1561-1573. doi:10.1056/NEJMra1510865
3. Nelson Textbook of Pediatrics Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 22nd ed. Elsevier; 2024.
4. GBD 2021 Sickle Cell Disease Collaborators. 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. Lancet Haematol. 2023;10(8):e585-e599. doi:10.1016/S2352-3026(23)00118-7
5. Piel FB, Patil AP, Howes RE, Nyangiri OA, Gething PW, Dewi M, et al. Global epidemiology of sickle haemoglobin in neonates: a contemporary geostatistical model-based map and population estimates. Lancet. 2013;381(9861):142-151. doi:10.1016/S0140-6736(12)61229-X
6. Hassan MK, Taha JY, Al-Naama LM, Widad NM, Jasim SN. Frequency of haemoglobinopathies and glucose-6-phosphate dehydrogenase deficiency in Basra. East Mediterr Health J. 2003;9(1-2):45-54.
7. Al-Jafar AJ, Al-Hussaini AA, Hassan MK. Hospitalization events among children and adolescents with sickle cell disease in Basra, Iraq. Anemia. 2015;2015:195469. doi:10.1155/2015/195469
8. Sadeq A, et al. Sonographic assessment of splenic manifestations in sickle cell disease patients and its relation to hematological parameters: a cross-sectional study in Basra, Iraq. Cureus. 2024;16(10):e72322. doi:10.7759/cureus.72322
9. Yaseen NT, Al-Mamoori HS, Hassan MK. Sickle β-globin haplotypes among patients with sickle cell anemia in Basra, Iraq: a cross-sectional study. Iraqi J Hematol. 2020;9(1):23-29. doi:10.4103/ijh.ijh_20_19
10. Jumaa DS, Al Hijaj BA, Dixen JZ. Erythrocytapheresis service in Basrah: safety and efficacy statistics. Eur J Med Health Res. 2024;2(5):318-326. Accessed May 27, 2026. Available at: https://ejmhr.com/index.php/journal/article/view/316
11. Kassim AA, Galadanci NA, Pruthi S, DeBaun MR. How I treat and manage strokes in sickle cell disease. Blood. 2015;125(22):3401-3410. doi:10.1182/blood-2014-09-551564
12. Ohene-Frempong K, Weiner SJ, Sleeper LA, Miller ST, Embury SH, Moohr JW, et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood. 1998;91(1):288-294.
13. 13.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;4(8):1554-1588. doi:10.1182/bloodadvances.2019001142
14. 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(8):699-710. doi:10.1056/NEJMoa1401731
15. 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;117(3):772-779. doi:10.1182/blood-2010-01-261123
16. Switzer JA, Hess DC, Nichols FT, Adams RJ. Pathophysiology and treatment of stroke in sickle-cell disease: present and future. Lancet Neurol. 2006;5(6):501-512. doi:10.1016/S1474-4422(06)70469-0
17. Adams RJ, 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. doi:10.1056/NEJMoa050460
18. Pegelow CH, Adams RJ, McKie V, Abboud M, Berman B, Miller ST, et al. Risk of recurrent stroke in patients with sickle cell disease treated with erythrocyte transfusions. J Pediatr. 1995;126(6):896-899. doi:10.1016/S0022-3476(95)70204-0
19. Powars D, Wilson B, Imbus C, Pegelow C, Allen J. The natural history of stroke in sickle cell disease. Am J Med. 1978;65(3):461-471. doi:10.1016/0002-9343(78)90772-6
20. Balkaran B, Char G, Morris JS, Thomas PW, Serjeant BE, Serjeant GR. Stroke in a cohort of patients with homozygous sickle cell disease. J Pediatr. 1992;120(3):360-366. doi:10.1016/S0022-3476(05)80897-2
21. Adams RJ, McKie VC, Hsu L, Files B, Vichinsky E, Pegelow C, 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. doi:10.1056/NEJM199807023390102
22. Lee MT, Piomelli S, Granger S, Miller ST, Harkness S, Brambilla DJ, et al. Stroke Prevention Trial in Sickle Cell Anemia (STOP): extended follow-up and final results. Blood. 2006;108(3):847-852. doi:10.1182/blood-2005-10-009506
23. Bernaudin F, Verlhac S, Arnaud C, Kamdem A, Chevret S, Hau I, et al. Impact of early transcranial Doppler screening and intensive therapy on cerebral vasculopathy outcome in a newborn sickle cell anemia cohort. Blood. 2011;117(4):1130-1140. doi:10.1182/blood-2010-06-293514
24. Ware RE, Davis BR, Schultz WH, Brown RC, Aygun B, Sarnaik S, et al. Hydroxycarbamide versus chronic transfusion for maintenance of transcranial Doppler flow velocities in children with sickle cell anaemia—TCD With Transfusions Changing to Hydroxyurea (TWiTCH): a multicentre, open-label, phase 3, non-inferiority trial. Lancet. 2016;387(10019):661-670. doi:10.1016/S0140-6736(15)01041-7
25. Ware RE, Helms RW; SWiTCH Investigators. Stroke With Transfusions Changing to Hydroxyurea (SWiTCH). Blood. 2012;119(17):3925-3932. doi:10.1182/blood-2011-11-392340
26. Helton KJ, Adams RJ, Kesler KL, Lockhart A, Aygun B, Driscoll C, et al. Magnetic resonance imaging/angiography and transcranial Doppler velocities in sickle cell anemia: results from the SWiTCH trial. Blood. 2014;124(6):891-898. doi:10.1182/blood-2013-12-545186
27. Wang WC, Ware RE, Miller ST, Iyer RV, Casella JF, Minniti CP, et al. Hydroxycarbamide in very young children with sickle-cell anaemia: a multicentre, randomised, controlled trial (BABY HUG). Lancet. 2011;377(9778):1663-1672. doi:10.1016/S0140-6736(11)60355-3
28. Schwartz J, Padmanabhan A, Aqui N, Balogun RA, Connelly-Smith L, Delaney M, et al. Guidelines on the use of therapeutic apheresis in clinical practice: evidence-based approach from the Writing Committee of the American Society for Apheresis: the seventh special issue. J Clin Apher. 2016;31(3):149-162. doi:10.1002/jca.21470
29. Padmanabhan A, Connelly-Smith L, Aqui N, Balogun RA, Klingel R, Meyer E, et al. Guidelines on the use of therapeutic apheresis in clinical practice: evidence-based approach from the Writing Committee of the American Society for Apheresis: the eighth special issue. J Clin Apher. 2019;34(3):171-354. doi:10.1002/jca.21705.
30. Sarode R, Matevosyan K, Rogers ZR, Burner JD, Rutherford CJ. Advantages of isovolemic hemodilution-red cell exchange therapy to prevent recurrent stroke in sickle cell anemia patients. J Clin Apher. 2011;26(4):200-207. doi:10.1002/jca.21203
31. Adams DM, Schultz WH, Ware RE, Kinney TR. Erythrocytapheresis can reduce iron overload and prevent the need for chelation therapy in chronically transfused pediatric patients. J Pediatr Hematol Oncol. 1996;18(1):46-50. doi:10.1097/00043426-199602000-00009
32. Hilliard LM, Williams BF, Lounsbury AE, Howard TH. Erythrocytapheresis limits iron accumulation in chronically transfused sickle cell patients. Am J Hematol. 1998;59(1):28- 35. doi:10.1002/(SICI)1096-8652(199809)59:1<28: AID-AJH6>3.0.CO;2-1
33. Kim HC. Red cell exchange: special focus on sickle cell disease. Hematology Am Soc Hematol Educ Program. 2014;2014(1):450-456. doi:10.1182/asheducation-2014.1.450
34. Lawson SE, Oakley S, Smith NA, Bareford D. Red cell exchange in sickle cell disease. Clin Lab Haematol. 1999;21(2):99-102. doi:10.1046/j.1365-2257.1999.00192.x
35. Tsitsikas DA, Sirigireddy B, Nzouakou R, Calvey A, Quinn J, Collins J, et al. Safety, tolerability, and outcomes of regular automated red cell exchange transfusion in the management of sickle cell disease. J Clin Apher. 2016;31(6):545-550. doi:10.1002/jca.21447
36. Verduzco LA, Nathan DG. Sickle cell disease and stroke. Blood. 2009;114(25):5117-5125. doi:10.1182/blood-2009-05-220921.
37. Brousse V, Buffet P, Rees D. The spleen and sickle cell disease: the sick(led) spleen. Br J Haematol. 2014;166(2):165-176. doi:10.1111/bjh.12950
38. Chou ST, Alsawas M, Fasano RM, Field JJ, Hendrickson JE, Howard J, et al. American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support. Blood Adv. 2020;4(2):327-355. doi:10.1182/bloodadvances.2019001143
39. Charache S, Terrin ML, Moore RD, Dover GJ, Barton FB, Eckert SV, et al. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. N Engl J Med. 1995;332(20):1317-1322. doi:10.1056/NEJM199505183322001
40. Steinberg MH, Barton F, Castro O, Pegelow CH, Ballas SK, Kutlar A, 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;289(13):1645-1651. doi:10.1001/jama.289.13.1645
41. Voskaridou E, Christoulas D, Bilalis A, Plata E, Varvagiannis K, Stamatopoulos G, 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(12):2354-2363. doi:10.1182/blood-2009-05-221333
42. Strouse JJ, Lanzkron S, Beach MC, Haywood C, Park H, Witkop C, et al. Hydroxyurea for sickle cell disease: a systematic review for efficacy and toxicity in children. Pediatrics. 2008;122(6):1332-1342. doi:10.1542/peds.2008-0441
43. Aljuburi G, Laverty AA, Green SA, Phekoo KJ, Banarsee R, Okoye NV, et al. Trends in hospital admissions for sickle cell disease in England, 2001/02-2009/10. J Public Health (Oxf). 2012;34(4):570-576. doi:10.1093/pubmed/fds035
44. Galadanci N, Wudil BJ, Balogun TM, Ogunrinde GO, Akinsulie A, Hasan-Hanga F, et al. Current sickle cell disease management practices in Nigeria. Int Health. 2014;6(1):23-28. doi:10.1093/inthealth/iht022
45. Panepinto JA, Bonner M. Health-related quality of life in sickle cell disease: past, present, and future. Pediatr Blood Cancer. 2012;59(2):377-385. doi:10.1002/pbc.24176
46. Schatz J, Brown RT, Pascual JM, Hsu L, DeBaun MR. Poor school and cognitive functioning with silent cerebral infarcts and sickle cell disease. Neurology. 2001;56(8):1109-11. doi:10.1212/wnl.56.8.1109. PMID: 11320190.