Sickle cell disease: regular blood transfusions in children with stroke may reduce risk.

Clinical bottom line (level 2b-)

  1. Patients with sickle cell disease and acute neurologic deficits who were considered at high risk and given transfusion therapy had no clear difference in further neurological events than those considered at low risk and given no transfusion.
Russell et al: Blood 1984; 63 (1): 162-169
Expires May 2003

The study

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

30 patients (aged range 18 months to 18 years, ?% male) sickle cell disease and acute neurologic deficits

Excluded if
  • refused consent


    • Control Group: (n = 7, 6 analysed): low risk group- no transfusion
    Experimental Group: (n = 23, 20 analysed): transfusion therapy- exchange transfusion of whole blood, or repeated transfusions of red cells until haemoglobin 12-14 g/dl and HbS<30% total HB. Repeated every 3-4 weeks so Hb 8-10 g/dl and HbS<30%.

    87% followed for 9 years

    The evidence

    Outcome Time to outcome CEREERRRR
    (95% CI)    		ARR
    (95% CI)    		NNT
    (95% CI)
    further transient ischaemic attack or stroke 9 years 1
    (16.7%)    		 3
    (15.0%)    		 10%
    (-610% to 89%)    		 1.67%
    (-32.0% to 35.3%)    		 60
    (NNT = 3 to infinity;
    NNH = 3 to infinity)

    Comments

    1. The groups were at different risks- not comparable. Transfusion may be of benefit for high risk patients, but this study cannot demonstrate this.
    2. 54% of strokes occurred with an accompanying illness (painful crises, viral illness, aplastic crises, pneumonia, dehydration).

    Citation

    1. Russell MO, Goldberg HI, Hodson A, et al: Effect of transfusion therapy on arteriographic abnormalities and on recurrence of stroke in sickle cell disease. Blood 1984; 63 (1): 162-169
    Contributor: Nick Shenker and Clare Wotton, May 2000
    Reviewer:

    Clinical Question.
    Patient sickle cell disease and acute neurologic deficit
    Intervention or Exposure transfusion therapy
    Comparison no transfusion
    Outcome further TIA or stroke.