Sickle cell disease: transfusion decreased hospital admissions.

Clinical bottom line (level 4)

  1. Children with sickle cell disease and cerebrovascular accidents or intractable pain who were given long-term blood transfusions, had less hospitalisations than previously.
  2. Hospitalisations due to infection, vaso-occlusive crises or acute chest syndrome were reduced with transfusion.
Styles and Vichinsky: Journal of Pediatrics 1994; 125: 909-911
Expires February 2003

The study

Case series with objective outcomes, not adjusted for confounding factors, not validated in an independent set of patients.
Setting: children's hospital, USA

17 patients (aged 4 to 16 years; mean 10.5, ?% male) sickle cell disease (confirmed by electrophoresis and family studies)- 13 had cerebrovascular accident and 4 had intractable pain
Note:
  • Patients acted as their own controls before and after transfusion.


    • Control Group: (n = 17, 17 analysed): pre-transfusion
    Experimental Group: (n = 17, 17 analysed): monthly blood transfusions of leucocyte-poor packed red blood cells to maintain pre transfusion haemoglobin S proportion of 30% and haemoglobin 10-12 g/dL. Iron chelation started with deferoxamine was given when ferritin reached 1000 µ g/L

    100% followed for 2 years

    The evidence

    Outcome Control Group
    (SD)    		 Experimental Group
    (SD)    		 Mean Difference
    (95% CI)
    total hospitalisations 236
    ()     		32
    ()     		p<0.001
    ( to )
    hospitalisations due to vaso-occlusive crises 150
    ()     		5
    ()     		p=0.003
    ( to )
    hospitalisations due to acute chest infection 62
    ()     		3
    ()     		p<0.001
    ( to )
    due to viral infection 83
    ()     		16
    ()     		p=0.01
    ( to )
    due to bacterial infection 16
    ()     		1
    ()     		p=0.02
    ( to )

  • Development of alloimmunization occurred in 3 of 17 patients.

    • Comments

    1. No patient developed hepatitis B, C or HIV.
    2. All patients required deferoxamine.
    3. It is unclear whether differences were due to other factors (children growing up, better management of sickle cell disease during the study period).

    Citation

    1. Styles LA, and Vichinsky E: Effects of a long-term transfusion regimen on sickle cell-related illnesses. Journal of Pediatrics 1994; 125: 909-911
    Contributor: Nick Shenker and Clare Wotton, February 2000
    Reviewer:

    Clinical Question.
    Patient sickle cell disease
    Intervention or Exposure post-transfusion
    Comparison pre-transfusion
    Outcome hospitalisations