SMJ Current Issue

Principles of blood transfusion in sickle cell anemia
  Hussaine H Al-Saeed,  Ahmed H Al-Salem
Saudi Medical Journal 2002; Vol.  (12): 1443-1448


Sickle cell anemia (SCA), which is due to homozygous inheritance of the hemoglobin S(HbS) variant, results from a single amino-acid substitution of valine for glutamic acid in the 6th position among the 146 amino acids of the hemoglobin b-chain. It is one of the common inherited hemoglobinopathies in the Kingdom of Saudi Arabia (KSA). The prevalence of hemoglobin AS (HbAS), and hemoglobin SS (HbSS) in KSA is 7.4% (0-25.9%) and 1.1% (0-5.3%), giving an overall HbS gene frequency of 0.047.1 The clinical spectrum of this disease is variable, commonly characterized by repeated acute painful episodes, increased vulnerability to infections, and certain pathological phenomena such as acute chest syndrome (ACS), cerebro-vascular strokes, hepatopathy, and multiorgan failure. Packed red blood cells (PRBC) transfusion in SCA was advocated as a therapy and prophylaxis as early as 1940s in acute painful episodes, pregnancy, leg ulcers, and anemia, but there have been debates; however, regarding the benefits and risks of blood transfusion (BT) in various presentations of SCA. One reason for this is that BT may adversely affect patients with SCA by increasing blood viscosity and precipitating vaso-occlusive crisis. These patients are acclimatized to normally live at Hb level between 8 and 10gm/dl, and injudicious BT may subsequently build up the hematocrit and the viscosity of their blood, leading to vaso-occlusive crisis.P>

The importance of BT as a therapy in patients with SCA, however, needs to be emphasized and guidelines towards safe BT need to be setup taking into consideration the risks, and benefits as well as long-term impact.2 This is especially so in this era when the life expectancy of patients with SCA is progressively increasing.P>

Pre-transfusion requirements.B>I>FONT> FONT>Patients with SCA are prone to several complications and at times unexpected medical problems occur and for these reasons they should be diagnosed early. The neonatal Hb identification for sickling hemoglobinopathies is of vast consequence to enable practitioners, at areas of high prevalence of the gene, to recognize those at risk at birth needs to be emphasized. As a baseline, the following should be conducted for each patient: a) Blood grouping, which should include: ABO, Rhesus, Duffy, Lewis, Kell, Kidd, and MNSs systems whenever possible. b) Red blood cell (RBC) transfusion must be from a compatible donor with matched ABO, C, c, D, E, e, and K, k c) To minimize the possibility of developing alloimmunity, RBC transfusion should have pre-stored leukocytes depletion through effective filter. d) For patients who develop alloantibodies, cross matching should include at least Fya, Fyb as well as those antigens implicated in the alloantibodies prior to further transfusion. e) Hepatitis B, hepatitis C, human immunodeficiency virus (HIV) serological assays, as well as hepatitis GB-C, transfusion transmitted virus (TTV) whenever possible. f) Vaccination against Hepatitis B, pneumococcal, Hemophilus Influenzae and meningococci.P>

Indications for blood transfusion in sickle cell anemia.B>I> The life span of sickle cells is only 15-20 days compared to 120 days for normal red cells, and as a result of this, most patients with SCA have moderate anemia. This may be exacerbated further in the event of complications such as splenic sequestration crisis, hyperhemolysis, infection and aplastic crisis. Whereas it is acceptable to use PRBC transfusion in symptomatic anemic patients who are prone to have a cardiovascular collapse, the indications for BT in patients with SCD are:P>

I. Definite indications.B>I> 1) Symptomatic anemia from any cause such as infection, hemolysis or aplastic crisis. 2) Acute splenic sequestration is defined as a sudden enlargement of the spleen size secondary to rapid sequestration of circ


From the Department of Medicine (Al-Saeed) and the Department of Surgery (Al-Salem), Qatif Central Hospital, Qatif, Kingdom of Saudi Arabia.

Address correspondence and reprint request to: Dr. Hussain H. Al-Saeed, PO Box 18100, Al-Jesh 31911, Qatif, Kingdom of Saudi Arabia. Tel. +966 (3) 8361000 Ext. 1013. Fax. +966 (3) 8360040. E-mail:



1.. El-Hazmi MA, Warsy AS, Al-Swailem AR, Al-Swailem AM, Bahakim HM. Sickle cell gene in the population of Saudi Arabia. Hemoglobin 1996; 20: 187-198.P>

2. Murphy MF, Wallington TB, Kelsy P, Boulton F, Lucas GF. Guidelines for clinical uses of red cell transfusion. Br J Haematol 2001; 113: 24-31.P>

3. Emond AM, Collis R, Darvill D, Higgs DR, Maude GH, Serjeant GR. Acute splenic sequestration in homozygous sickle cell disease: Natural history and management. J Pediatr 1985; 107: 201-206. P>

4. Rao SP, Miller ST, Cohen BJ. Transient aplastic crisis in patient with sickle cell disease: B19 Parvovirus studies during a 7-year-period. Am J Dis Child 1992; 146: 1328-1330.P>

5. Serjeant GR, Serjeant BE, Thomas PW, Anderson MJ, Patou G, Pattison JR. Human parvovirus infection in homozygous sickle cell disease. Lancet 1993; 341: 1237-1240. P>

6. Frickhofen N, Abkowitz JL, Safford M, Berry JM, Antunez-de-Mayolo J, Astrow A et al. Persistent B19 parvovirus infection in patient with human immunodeficiency virus type 1: a treatable cause of anemia in AIDS. Ann Intern Med 1990; 113: 926-933.P>

7. Castro O, Brambilla DJ, Thorington B, Reindorf CA, Scott RB, Gillette P et al. The acute chest syndrome in sickle cell disease: Incidence and risk factors. The cooperative Study of sickle cell disease. Blood 1994; 84: 643-649.P>

8. Vichinsky EP, Styles LA, Colangelo LH, Wright EC, Castro O, Nickerson B. Acute chest syndrome in sickle cell disease: clinical presentation and course. Cooperative Study of Sickle Cell Disease. Blood 1997; 89: 1787-1792.P>

9. Emre U, Miller ST, Gutierez M, Steiner P, Rao SP, Rao M. Effect of transfusion in acute chest syndrome of sickle cell disease. J Pediatr 1995; 127: 901-904.P>

10. Mallouh AA, Asha M. Beneficial effect of blood transfusion in children with sickle cell chest syndrome. Am J Dis Child 1988; 142: 178-182.P>

11. Vichinsky EP, Neumayr LD, Earles AN, Williams R, Lennette ET, Dean D 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; 342: 1855-1865.P>

12. Powars D, Wilson B, Imbus C, Pegelow C, Allen J. The natural history of stroke in sickle cell disease. Am J Med 1978; 65: 461-471.P>

13. Ohene-Frempong K, Weiner SJ, Sleeper LA, Miller ST, Embury S, Moohr JW et al. Cerebrovascular accidents in sickle cell disease: Rates and risk factors. Blood 1998; 91: 288-294.P>

14. Russell MO, Goldberg HI, Hodson A, Kim HC, Halus J, Reivich M et al. Transfusion therapy for cerebrovascular abnormalities in sickle cell disease. Blood 1984; 63: 162-169.P>

15. Ohene-Frempong K. Stroke in sickle cell disease demographic, clinical, and therapeutic considerations. Semin Hematol 1991; 28: 213-219.P>

16. Gaston MH, Vector JI, Woods G, Pegelow C, Kelleher J, Presbury G et al. Prophylaxis with oral penicillin in children with sickle cell anemia: a randomized trial. N Engl J Med 1986; 314: 1593-1599.P>

17. Zarkowsky HS, Gallagher D, Gill FM, Wang WC, Falletta JM, Lande WM et al. Bacteremia in sickle hemoglobinopathies. J Pediatr 1986; 109: 579-585.P>

18. Magnus SA, Hambleton IR, Moosdeen F, Serjeant GR. Recurrent infections in sickle cell disease. Arch Dis Child 1999; 80: 537-541.P>

19. 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: 155-162.P>

20. Vichinsky EP, Neumayr LD, Haberkern C, Earles AN, Black D, Koshy M 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: 206-213.P>

21. Vichinsky EP, Neumayr LD, Haberkern C, Earles AN, Eckman J, Koshy M et

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