Sickle cell crisis: recurrence of splenic sequestration was common.

Clinical bottom line (level 4)

  1. Nearly two thirds of children with splenic sequestration had recurrence.
  2. Nearly two thirds of children who had splenic sequestration received a splenectomy eventually.
Kinney et al: Journal of Pediatrics 1990; 117 (2): 194-199
Expires February 2003

The study

Case series with objective outcomes, not adjusted for confounding factors, not validated in an independent set of patients.

Setting: sickle cell centre, USA

23 patients (aged range 0.5 to 9.5 years; mean 3, 61% male) children with sickle cell disease having splenic sequestration crisis (fall in haemoglobin of >2 g/dL, with an enlarged spleen and evidence of bone marrow erythroid activity)

Excluded if
  • anaemia secondary to aplastic crisis
  • immune-mediated haemolysis
  • iron deficiency


    • Patients were given any of: prompt splenectomy (4 patients; mean age 2.8); careful observation without additional therapy (7; mean age 2.8); short term transfusion (12; mean 1.7).

    100% followed for 1 to 7 years
    Outcomes studied:
  • eventually having splenectomy
  • under careful observation who had a further sequestration crisis
  • given transfusion and 7 had a further sequestration crisis during transfusion
  • had transfusion and sequestration crisis post-transfusion

    • 96% of patients had a transfusion initially.

    The evidence

    outcome time to outcome number of patients/total number %
    (95% CI)
    eventually having splenectomy 1 to 7 years 14/23 60.9%
    (40.9% to 80.8%)
    under careful observation who had a further sequestration crisis 1 to 7 years 13/23 56.5%
    (36.3% to 76.8%)
    given transfusion and 7 had a further sequestration crisis during transfusion 1 to 7 years 7/23 30.4%
    (11.6% to 49.2%)
    had transfusion and sequestration crisis post-transfusion 1 to 7 years 9/23 39.1%
    (19.2% to 59.1%)

    Comments

    1. Study was too small and compares unsimilar groups.

    Citation

    1. Kinney TR, Ware RE, Schultz WH, et al: Long-term management of splenic sequestration in children with sickle cell disease. Journal of Pediatrics 1990; 117 (2): 194-199
    Contributor: Chris Ball and Clare Wotton, February 2000
    Reviewer:

    Clinical Question.
    Patient sickle cell disease
    Intervention or Exposure various treatments
    Outcome require splenectomy eventually