Sickle cell disease: about 7% of patients died within 2 years.

Clinical bottom line (level 1b)

  1. About 7% of patients with sickle cell disease will died within 2 years.
  2. Patients aged over 20 years were at increased risk of death at two years if they had:
    • low foetal haemoglobin
    • acute chest syndrome
    • renal failure
    • seizures
    • high white cell count
Platt et al: New England Journal of Medicine 1994; 330 (23): 1639-1644
Expires June 2003

The study

Prospective cohort study with objective outcomes, adjusted for confounding factors, not validated in an independent set of patients.

Setting: 23 clinical centres, USA

3764 patients (aged range birth to 66 years; mean 13.5, 51% female) sickle cell disease

Factors studied:
  • death




    • Proportional-hazards regression was used to adjust for confounding factors.

    ?100% followed for 2 years
    Outcomes studied:
  • death

    • The evidence

    outcome time to outcome number of patients/total number %
    (95% CI)
    death 2 years 249/3764 6.62%
    (5.82% to 7.41%)

    • Median age at death was 42 years for males and 48 years for females.
    • In patients 20 years or older, death was more likely if they had:
      • low foetal haemoglobin, p<0.001
      • acute chest syndrome, p=0.005
      • renal failure, p=0.03
      • seizures, p=0.04
      • high white cell count, p=0.01

    Comments

    1. The number of deaths in patients 20 years or older was not given, and no odds ratios were given for the risk factors.
    2. No prognostic factors for death in under 20 year olds were suggested.

    Citation

    1. Platt OS, Brambilla DJ, Rosse WF, et al: Mortality in sickle cell disease: life expectancy and risk factors for early death. New England Journal of Medicine 1994; 330 (23): 1639-1644
    Contributor: Clare Wotton and Musab Hayatli, June 2000
    Reviewer:

    Clinical Question.
    Patient sickle cell disease
    Intervention or Exposure prognostic factors
    Outcome death