Sickle cell anaemia: older age, high surgical risk and history of some disease increased the risk of complications following surgery.

Clinical bottom line (level 1b-)

  1. Patients with sickle cell disease and having surgery were at an increased risk of painful crises if they:
    • were aged 20 years or older (NNF = 3 for 30 days)
    • had 5 or more hospital attendances (NNF = 6 for 30 days)
  2. Patients were at an increased risk of acute chest syndrome if they:
    • had a surgical risk of 2 or 3 (NNF = 7 for 30 days)
    • had a history of pulmonary disease (NNF = 14 for 30 days)
  3. Patients were at an increased risk of any two or three complications if they:
    • were aged 20 years or older (NNF = 7 for 30 days)
    • had a history of CNS disease (NNF = 4 for 30 days)
    • alloimmunization (NNF = 7 for 30 days)
    • surgical risk 2 or 3 (NNF = 7 for 30 days)
  4. Patients were at an increased risk of transfusion complications if they:
    • had any complication (NNF = 10 for 30 days)
    • had new alloantibodies (NNF = 9 for 30 days)
Vichinsky et al: New England Journal of Medicine 1995; 333 (4): 206-213
Expires May 2003

The study

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

Setting: 36 hospitals, USA

551 patients (aged 40% aged 0-9; 35% aged 10-19 years, 51% male) sickle cell anaemia undergoing elective surgery

Excluded if
  • transfusion within last three months



    • Factors studied:
  • painful crises, acute chest syndrome, any two or three complications, transfusion reaction
  • aged = 20 years
  • = 5 hospital attendances ever
  • surgical risk of 2 or 3
  • history of pulmonary disease
  • aged = 20 years
  • history of central nervous disease
  • alloimmunization
  • surgical risk 2 or 3
  • any complication
  • new alloantibody


    • Patients were randomised to aggressive or conservative transfusion.

    Multivariate analysis was used to adjust for confounding factors.

    100% followed for 30 days
    Outcomes studied:
  • painful crisis
  • acute chest syndrome
  • any two or three complications
  • transfusion complications

    • The evidence

    outcome time to outcome number of patients/total number %
    (95% CI)
    painful crisis 30 days 35/604 5.79%
    (3.93% to 7.66%)
    acute chest syndrome 30 days 63/604 10.4%
    (7.99% to 12.9%)
    any two or three complications 30 days 198/604 32.8%
    (29.0% to 36.5%)
    transfusion complications 30 days 63/604 10.4%
    (7.99% to 12.9%)

    prognostic factor for
    painful crisis     		time to outcome control rate (%) adjusted OR
    (95% CI)    		 NNF+
    (95% CI)
    aged = 20 years 30 days 35/604
    (5.79%)     		10.2
    (2.05 to 51.0)     		3
    (1 to 19)
    = 5 hospital attendances ever 30 days 35/604
    (5.79%)     		5.00
    (1.33 to 19.0)     		6
    (2 to 57)

    prognostic factor for
    acute chest syndrome     		time to outcome control rate (%) adjusted OR
    (95% CI)    		 NNF+
    (95% CI)
    surgical risk of 2 or 3 30 days 63/604
    (10.4%)     		2.97
    (1.30 to 6.81)     		7
    (3 to 37)
    history of pulmonary disease 30 days 63/604
    (10.4%)     		1.86
    (1.07 to 3.23)     		14
    (6 to 154)

    prognostic factor for
    any two or three complications     		time to outcome control rate (%) adjusted OR
    (95% CI)    		 NNF+
    (95% CI)
    aged = 20 years 30 days 198/604
    (32.8%)     		1.80
    (1.04 to 3.81)     		7
    (3 to 115)
    history of central nervous disease 30 days 198/604
    (32.8%)     		2.76
    (1.48 to 5.12)     		4
    (3 to 11)
    alloimmunization 30 days 198/604
    (32.8%)     		1.88
    (1.15 to 3.08)     		7
    (4 to 32)
    surgical risk 2 or 3 30 days 198/604
    (32.8%)     		1.76
    (1.08 to 2.89)     		7
    (4 to 58)

    prognostic factor for
    transfusion complications     		time to outcome control rate (%) adjusted OR
    (95% CI)    		 NNF+
    (95% CI)
    any complication 30 days 63/604
    (10.4%)     		2.15
    (1.23 to 3.77)     		10
    (5 to 48)
    new alloantibody 30 days 63/604
    (10.4%)     		2.33
    (1.21 to 4.49)     		9
    (4 to 52)

    Citation

    1. Vichinsky EP, Haberkern CM, Neumayr L, et al: A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease. New England Journal of Medicine 1995; 333 (4): 206-213
    Contributor: Chris Ball and Clare Wotton, May 2000
    Reviewer:

    Clinical Question.
    Patient sickle cell anaemia
    Intervention or Exposure prognostic factors
    Outcome serious complications