Blood transfusion in sickle cell disease

Current Opinion in Hematology

Volumn 3, Issue 6, 1996, Pages 485-491

  Davies, Sally C ^a  

^a CENTRAL MIDDLESEX HOSPITAL   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

BLOOD BANK; BLOOD TRANSFUSION; BLOOD TRANSFUSION REACTION; BONE MARROW TRANSPLANTATION; CEREBROVASCULAR DISEASE; CLINICAL TRIAL; ERYTHROCYTE TRANSFUSION; EXCHANGE BLOOD TRANSFUSION; HUMAN; KIDNEY DISEASE; LIVER DISEASE; PREGNANCY; PRIAPISM; PRIORITY JOURNAL; REVIEW; SICKLE CELL ANEMIA; THORAX DISEASE; TREATMENT OUTCOME;

EID: 0029857259     PISSN: 10656251     EISSN: None     Source Type: Journal    
DOI: 10.1097/00062752-199603060-00015     Document Type: Review

References (67)

1. Platt OS, Brambilla DJ, Rosse PF, Milner PF, Castro O, Steinberg MH, Klug PP: Mortality in sickle cell disease: life expectancy and risk factors for early death. N Engl J Med 1994, 330:1639-1644.
2. Davies SC, McWilliam AC, Hewitt PE, Devenish A, Brozovic M: Red cell alloimmunization in sickle cell disease. Br J Haematol 1986, 63:241-245.
3. Vichinsky EP, Earles A, Johnson RA, Hoag MS, Williams A, Lubin B: Alloimmunization in sickle cell anemia and transfusion of racially unmatched blood. N Engl J Med 1990, 322:1617-1621.
4. Rosse WF, Gallagher D, Kinney TR, Castro O, Dosik H, Moohr J, Wang W, Levy PS, Cooperative Study of Sickle Cell Disease: Transfusion and alloimmunization in sickle cell disease. Blood 1990, 76:1431-1437.
5. Cummins D, Webb G, Shah N, Davies SC: Delayed haemolytic transfusion reactions in patients with sickle cell disease. Postgrad Med J 1991, 67:689-691.
6. Heddle NM, Soutar RL, O'Hoski PL, Singer J, McBride JA, Ali MAM, Kelton JG: A prospective study to determine the frequency and clinical significance of alloimmunization post-transfusion. Br J Haematol 1995, 91:1000-1005. A large prospective study of posttransfusion alloimmunization to erythrocyte antigens, with no reported racial difference between the donors and recipients, demonstrated alloantibodies in 2.6% of the patients. Only one of the 2490 patients (0.05%) had clinical evidence of a DHTR, supporting the recommended policies.
7. Tahhan HR, Holbrook CT, Braddy LR, Brewer LD, Christie JD: Antigen-matched donor blood in the transfusion management of patients with sickle cell disease. Transfusion 1994, 34:562-569.
8. Freidman DF, Kim HC, Manno CS: Hyperhaemolysis associated with red cell transfusion in sickle cell disease. Transfusion 1993, 33(suppl):14.
9. Cullis JO, Win N, Dudley JM, Kaye T: Post-transfusion hyperhaemolysis in a patient with sickle cell disease: use of steroids and intravenous immunoglobulin to prevent further red cell destruction. Vox Sang 1995, 69:355-357.
10. Wong W, Powars DR, Operskalski EA, Hasset J, Parker JW, Sarnaik S, Pegelow CH, Hilgartner MW, Johnson CS, Zhou Y, et al.: Blood transfusions and immunophenotypic alterations of lymphocyte subsets in sickle cell anemia. Blood 1995, 85:2091-2097.
11. Jan K, Usami S, Smith JA: Effects of transfusion on rheological properties of blood in sickle cell anemia. Transfusion 1982, 22:17-20.
12. Keidan AJ, Marwah SS, Vaughan GR, Franklin IM, Stuart J: Painful sickle cell crises precipitated by stopping prophylactic exchange transfusions. J Clin Pathol 1987, 40:505-507.
13. Rackoff WR, Ohene-Frempong K, Month S, Scott JP, Neahring B, Cohen AR: Neurologic events after partial exchange transfusion for priapism in sickle cell disease. Pediatrics 1992, 120:882-885.
14. Seigel JF, Rich MA, Brock WA: Association of sickle cell disease, priapism, exchange transfusion and neurological events: Aspen syndrome. J Urology 1993, 150:1480-1482.
15. Hasegawa S, Hiruma H, Uyesaka N, Noguchi CT, Schechter AN, Rodgers GP: Filterability of mixtures of sickle and normal erythrocytes. Am J Hematol 1995, 50:91-97. Erythrocyte deformability has been measured by filtration through 3 μm pores in nickel mesh. Filterability correlates linearly with the amount of HbS polymerization, and the percentage of sickle cells explains more than 77% of the variation in filtration, in the absence of dense dehydrated cells. This is an important in vitro study having implications regarding targets for exchange transfusion therapy. It also presents new and fascinating evidence of a reduction of immature reticulocytes in exchange-transfused patients, as compared with nontransfused patients, which may explain some of the early clinical improvement in patients with exchange transfusion.
16. Cohen AR, Martin MB, Silber JH, Kim HC, Ohene-Frempong K, Schwartz E: A modified transfusion program for prevention of stroke in sickle cell disease. Blood 1992, 79:1657-1661.
17. Kim HC, Dugan NP, Silber JH, Martin MB, Schwartz E, Ohene-Frempong K, Cohen A: Erythrocytapheresis therapy to reduce iron overload in chronically transfused patients with sickle cell disease. Blood 1994, 83:1136-1142.
18. Govoni M, Lodi GL, Lunghi M: Red cell exchange transfusion in severe HbS/beta-thalassemia double heterozygosis. Vox Sang 1995, 68:248.
19. King SD, Dodd RY, Haynes G, Winter HA, Sullivan MT, Serjeant GR, Choo-Kang E, Michael E: Prevalence of antibodies to hepatitis C virus and other markers in Jamaica. West Indian Med J 1995, 44:55-57. The prevalence of antibodies to hepatitis in Jamaica is low (0.3%) in blood donors but was more than seven times higher in the multiply transfused patients with sickle cell disease.
20. al Mahroos FT, Ebrahim A: Prevalence of hepatitis B, hepatitis C, and human immune deficiency virus molecules among patients with hereditary haemolytic anemia. Ann Trop Pediatr 1995, 15:121-128. In 191 multiply transfused patients in Bahrain, 40% were seropositive for hepatitis C virus antibodies, 20.9%, for hepatitis B virus, and 1.6% for HIV; as compared with one patient seropositive for only hepatitis C virus antibody in the nontransfusion group (n = 51).
21. Cohen BJ, Field AM, Gudnadottir S, Beard S, Barbara AJ: Blood donor screening for parvovirus B19. J Virol Method 1990, 30:233-238.
22. Yoto Y, Kudoh T, Haseyama K, Suzuki N, Oda T, Katoh T, Takahashi C, Sekigucji S, Chiba S: Incidence of human parvovirus B19 DNA detection in blood donors. Br J Haematol 1995, 91:1017-1018. In Japan, 0.6% of donors tested positive for B19 DNA, and five of the six samples also tested positive for anti-B19 IgM, indicating an acute phase of infection.
23. Francis RB: Platelets, coagulation, and fibrinolysis in sickle cell disease: their possible role in vascular occlusion. Blood Coagul Fibrinol 1991, 2:341-353.
24. Peters M, Plaat BEC, ten Cate H, Wolters HJ, Weening RS, Brandjes DPM: Enhanced thrombin generation in children with sickle cell disease. Thromb Hemost 1994, 71:169-172.
25. Abdul-Rauf A, Gauderer M, Chiarucci K, Berman B: Long-term central venous access in patients with sickle cell disease. J Pediatr Hematol Oncol 1995, 17:141-145.
26. Wolters HJ, ten Cate H, Thomas LLM, Brandjes DPM, van der Ende A, van der Heiden Y, van Epsa LWS: Low-intensity oral anticoagulation in sickle cell disease reverses the prethrombotic state: promises for treatment? Br J Haematol 1995, 90:715-717. These authors showed normalization of prothrombin fragments I and II in patients with sickle cell disease using acenocoumarol; they achieved a median international normalized ratio of 1.64 (range, 1.18 to 2.2), demonstrating that low-intensity oral anticoagulation normalizes the hypercoagulability in sickle cell disease.
27. Yeghen T, Benjamin S, Boyd O, Pumphrey C, Bevan DH: Sickle cell anemia, right atrial thrombosis, and the antiphospholipid antibody. Am J Hematol 1995, 50:46-48.
28. Rees DC, Cox M, Clegg JB: World distribution of factor V Leiden. Lancet 1995, 346:1133-1134.
29. Davies SC, Win AA, Luce PJ, Riordan JF: Acute chest syndrome in sickle cell disease. Lancet 1984, 36-38.
30. Gray A, Anionwu EN, Davies SC, Brozovic M: Mortality in sickle cell disease: the experience of a British centre. J Clin Pathol 1991, 44:459-463.
31. Emre U, Miller ST, Gutieriez M, Steiner P, Rao SP, Rao M: Effect of transfusion in acute chest syndrome of sickle cell disease. J Pediatr 1995, 127:901-904.
32. 2 levels in acute chest syndrome of sickle cell disease. Blood 1996, 87:2573-2578.
33. Powars DR, Weidman JA, Odom-Maryon T, Niland JC, Johnson C: Sickle cell chronic lung disease: prior morbidity and the risk of pulmonary failure. Medicine 1988, 67:66-76.
34. Charache S, Lubin B, Reid CD: Stroke. In Management and Therapy of Sickle Cell Disease, edn 3. Edited by Charache S, Lubin B, Reid CD. Washington, DC: US Department of Health; 1995:53-58. The most comprehensive advice on how to treat patients with sickle cell disease.
35. Pegelow CH, Adams RJ, McKie V, Abboud M, Berman B, Miller ST, Olivieri N, Vichinsky E, Wang W, Brambilla D: Risk of recurrent stroke in patients with sickle cell disease treated with erythrocyte transfusions. J Pediatr 1995, 126:896-899.
36. Wang WC, Kovnar EH, Tonkin IL, Mulhern RK, Langston JW, Day SW, Schell MJ, Wilimas JA: High risk of recurrent stroke after discontinuance of five to twelve years of transfusion therapy in patients with sickle cell disease. J Pediatr 1991, 118:377-382.
37. Miller ST, Jensen D, Rao SP: Less intensive long-term transfusion therapy for sickle cell anemia and cerebrovascular accident. J Pediatr 1992, 120:54-57.
38. Miller ST, Rao SP, Dunn EK, Glassberg KI: Priapism in children with sickle cell disease. J Urol 1995, 154:844-847.
39. Stephan JL, Merpit-Gonon E, Richard O, Raymond-Ravni C, Freycon F: Fulminant liver failure in a 12-year old girl with sickle cell anemia: favourable outcome after exchange transfusions. Eur J Pediatr 1995, 154:469-471.
40. Shao SH, Orringer EP: Sickle cell intrahepatic cholestasis: approach to a difficult problem. Am J Gastroenterol 1994, 90:2048-2050.
41. Lang T, Berquist WE, So SK, Cox KL, Rich EJ, Vichinsky EP, Concepcion W, Esquivel CO: Liver transplantation in a child with sickle cell anemia. Transplantation 1995, 59:1490-1492.
42. Koshy M, Burd L, Wallace D, Moawad A, Baron J: Prophylactic red-cell transfusions in pregnant patients with sickle cell disease. N Engl J Med 1988, 319:1447-1452.
43. Morrison JC, Morrison FS, Floyd RC, Roberts WE, Hess LW, Wiser WL: Use of continuous flow erythrocytapheresis in pregnant patients with sickle cell disease. J Clin Apheresis 1991, 6:224-229.
44. Howard RJ, Tuck SM, Pearson TC: Optimal haematocrit and haemoglobin S levels in pregnant women with sickle cell disease. Clin Lab Haematol 1995, 17:157-161. This study examining the rheology of transfusion in pregnant women with sickle cell disease indicates that potential oxygen delivery to the fetus only approaches normal at very low levels of HbS (< 20%). Based on data from 17 pregnant patients, the authors suggest an optimal packed cell volume during pregnancy of 0.26, although previous authors have calculated higher optimal hematocrits.
45. Vichinsky EP, Haberkern CM, Neumayr L, Earles AN, Black D, Koshy M, Pegelow C, Abboud M, Ohene-Frempong K, Iyer RV, 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, 333:206-213. A good and timely study demonstrating how multicenter randomized controlled trials should be performed in patients with sickle cell disease. Patients undergoing a total of 604 operations were randomly assigned to either exchange transfusion with the object of reducing the HbS below 30%, or additive transfusion with the object of raising the hemoglobin to 10 g/dL. (In practice, patients who were exchange transfused had average HbS levels of 31% and hemoglobins of 11 g/dL; those receiving additive transfusions had hemoglobins averaging 10.6 g/dL and HbS levels of 59%.) The conservative approach resulted in only half as many transfusion-related complications as the exchange regime, although it was equally effective in preventing perioperative complications. Acute chest syndrome occurred in 10 patients from each group, and painful crisis occurred in four exchange-transfused patients and seven patients receiving an additive transfusion. Fevers occurred in seven exchange-transfused patients and five patients receiving an additive transfusion, and neurologic events and renal complications occurred in not more than one patient from each group.
46. Koshy M, Weiner SJ, Miller ST, Sleeper LA, Vichinsky E, Brown AK, Khakoo Y, Kinney TR, Cooperative Study of Sickle Cell Disease: Surgery and anesthesia in sickle cell disease. Blood 1995, 65:3676-3684. One thousand seventy-nine surgical procedures were carried out on 7171 patients, with an overall mortality of 1.1% within 30 days of the procedure. The majority of the patients received transfusions that appeared to reduce postoperative sickle pain and acute chest syndrome, but not to unduly influence the development of fever. This study also demonstrated that postoperative complications after surgery were more frequent in patients who received regional rather than general anaesthesia.
47. Hatley RM, Christ D, Howell CF, Herline AJ, Gadacz TR: Laparoscopic cholecystectomy in children with sickle cell disease. Am Surg 1995, 61:169-71.
48. Griffin TC, Buchanan GR: Elective surgery in children with sickle cell disease without preoperative blood transfusion. J Pediatr Surg 1993, 28:681-685.
49. Baruchel S, Delifer JC, Sigalet D, Hong A, Oudjhane K, Chen MF: Pseudomembranous colitis in sickle cell disease responding to exchange transfusion. J Pediatr 1992, 121:915-917.
50. Buchanan GR, Rogers ZR: Conservative versus aggressive transfusion regimens in sickle cell disease. N Engl J Med 1995, 333:1641-1642.
51. Davies SC, Roberts IAG: Bone marrow transplant for sickle cell disease: an update. Arch Dis Child 1996, in press.
52. Mentzer WC, Heller S, Pearle PR, Hackney E, Vichinsky E: Availability of related donors for bone marrow transplantation in sickle cell anemia. Am J Pediatr Hematol Oncol 1994, 16:27-29.
53. Davies SC, Wonke B: The management of hemoglobinopathies. Baillieres Clin Haematol 1991, 4:361-390
54. Issaragrish S, Visuthisakchai S, Suvatte V, Tanphaichite VS, Chandanayingyong D, Schreiner T, Konokpongsakdi S, Sinitanaratkul N, Piankijagum A: Brief report: transplantation of cord-blood stem cells into a patient with severe thalassemia. N Engl J Med 1995, 332:367.
55. Touraine JL, Rudrant D, Rebaud A, Roncarolo MG, Laplace S, Gebuhrer L, Betuel H, Frappaz D, Freycon F, Zabot MT, et al.: In utero transplantation of stem cells in humans: immunological aspects and clinical follow up. Bone Marrow Transplant 1992, (suppl 1):121-126.
56. Kohn DB, Weinberg KI, Nolta JA, Heiss LN, Lenarsky C, Crooks GM, Hanley ME, Annett G, Brooks JS, El-Khoureiy A, et al.: Engraftment of gene-modified umbilical cord blood cells in neonates with adenosine deaminase deficiency. Nature Med 1995, 1:1017-1023.
57. McCune SL, Reilly MP, Chomo MJ, Asakura T, Townes TM: Recombinant human hemoglobins designed for gene therapy of sickle cell disease. Proc Natl Acad Sci U S A 1994, 91:9852-9856.
58. 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, 332:1317-1322. This double-blind, randomized clinical trial is a landmark study of the efficacy of hydroxyurea in reducing the frequency of painful crisis in sickle cell disease. Not only was there a significant amelioration in the clinical picture, with a reduction in painful crises, but patients assigned to hydroxyurea also had fewer episodes of acute chest syndrome and fewer blood transfusions. A number of questions remain unanswered, and understanding of the method of action of hydroxyurea in sickle cell disease as well as optimal dosage regimens remains uncertain.
59. Vichinsky EP, Lubin BH: A cautionary note regarding hydroxyurea in sickle cell disease. Blood 1994, 83:1124-1128.
60. Ware RE, Steinberg MH, Kinney TR: Hydroxyurea: an alternative to transfusion therapy for stroke in sickle cell anemia. Am J Hematol 1995, 50:140-143.
61. Goldberg MA, Brugnara C, Dover GJ, Schapira L, Charache S, Bunn HF: Treatment of sickle cell anemia with hydroxyurea and erythropoietin. N Engl J Med 1990, 323:366-372.
62. Rodgers GP, Dover GJ, Uyesaka N, Noguchi CT, Schechter AN, Nienhuis AW: Augmentation by erythropoietin of the fetal-hemoglobin response to hydroxyurea in sickle cell disease. N Engl J Med 1993, 328:73-80.
63. Perrine SP, Ginder GD, Faller DV, Dover GJ, Ikuta T, Witkowska HE, Cai S, Vichinsky EP, Olivieri NF: A short-term trial of butyrate to stimulate fetal-globin-gene expression in the β-globin disorders. N Engl J Med 1993, 328:81.
64. Sher GD, Ginder GD, Little J, et al.: Extended therapy with intravenous arginine butyrate in patients with β-hemoglobinopathies. N Engl J Med 1995, 332:1606-1610.
65. Dover GJ, Brusilow S, Charache S: Induction of fetal hemoglobin production in subjects with sickle cell anemia by oral sodium phenylbutyrate. Blood 1994, 84:339-343.
66. Wood AE, Nathwani A, Thorpe SJ, Davies SC: Induction of fetal hemoglobin in individuals with sickle call anemia by sodium valproate [abstract]. Br Soc Haematol 1996.
67. de Franceschi L, Rouyer-Fessard P, Alper SL, Joualt H, Brugnara C, Beuzard Y: Combination therapy of erythropoietin, hydroxyurea, and clotrimazole in a β thalassemic mouse: a model for human therapy. Blood 1996, 97:1188-1195.