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

 

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: alsaeedhha@hotmail.com

 

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