Sickle cell crisis: splenectomy increased haemoglobin and red blood cell survival.

Clinical bottom line (level 4)

  1. Jamaican children with homozygous sickle cell disease who had a splenectomy had an increase in haemoglobin and red blood cell survival.
Emond et al: Lancet 1984; 1: 88-90
Expires February 2003

The study

Case series with unblinded, unobjective outcomes, not adjusted for confounding factors, not validated in an independent set of patients.
Setting: university hospital, Jamaica

60 patients (aged mean 9 to 62 months; median 18, 57% female) Jamaican children with homozygous sickle cell disease who had a splenotomy between 1954 and 1982
Note:
  • 23% of patients had splenectomy for acute splenic sequestration; 77% for chronic hypersplenism


    • Control Group: (n = 60, 37 analysed): pre-splenectomy
    Experimental Group: (n = 60, 37 analysed): post-splenectomy

    62% followed for 6 years

    The evidence

    Outcome Control Group
    (SD)    		 Experimental Group
    (SD)    		 Mean Difference
    (95% CI)
    haemoglobin g/dL (n=37) 5.00
    (1.00)     		8.50
    (0.90)     		-3.50
    (-3.94 to -3.05)
    red blood cell survival (half life in day) (n=10) 3.90
    (3.80)     		10.1
    (2.50)     		-6.20
    (-7.69 to -4.71)
    irreversible sickle cells (%) (n=17) 1.56
    (0.93)     		5.21
    (3.51)     		-3.65
    (-4.84 to -2.46)

  • 8% of patients had acute chest syndrome.
  • Three patients died- none had received antibiotic prophylaxis.

    • Comments

    1. The trial was small and follow-up was very poor.
    2. Current prophylaxis has reduced the problem of post splenectomy sepsis.
    3. A partial splenectomy has been done in some centres, though technically this is a difficult operation

    Citation

    1. Emond AM, Morais P, Venugopal S, et al: Role of splenectomy in homozygous sickle cell disease in childhood. Lancet 1984; 1: 88-90
    Contributor: Chris Ball and Clare Wotton, February 2000
    Reviewer: Sudha Chaudhari

    Clinical Question.
    Patient sickle cell disease
    Intervention or Exposure pre-splenectomy
    Comparison post-splenectomy
    Outcome blood properties