Sickle cell disease: a quarter had an episode of acute splenic sequestration.

Clinical bottom line (level 2c)

A quarter of children with homozygous sickle cell disease had an episode of acute splenic sequestration within 10 years.
A sixth of those with acute splenic sequestration died.

Emond et al: Journal of Pediatrics 1985; 107 (2): 201-206

Expires April 2003

The study

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

Setting: maternity hospital, Jamaica

314 patients (aged range 2 to 10 years, ?% male) homozygous sickle cell disease, from 100,000 consecutive normal deliveries screened

97% followed for 10 years
Outcomes studied:

acute splenic sequestration drop of haemoglobin by 2 g/dl and marrow response in presence of acutely enlarging spleen

mortality with sequestration

recurrence

An education program to allow mothers to detect early sequestration crisis was started in 1977.

The evidence

outcome	time to outcome	number of patients/total number	% (95% CI)	NNF (95% CI)
acute splenic sequestration	10 years	89/308	29% (24% to 34%)	3 (3 to 4)
mortality with sequestration	10 years	13/89	15% (7.3% to 22%)	7 (5 to 14)
recurrence	10 years	38/74	51% (40% to 63%)	2 (2 to 3)

Four patients had splenectomy.
There a total of 42 deaths in the cohort (13 were sequestration).

Comments

Although ASS can be treated effectively with packed cells transfusions, its delayed recognition and/or treatment may result in a fatal outcome

Citation

Emond AM, Collis R, Darvill D, et al: Acute splenic sequestration in homozygous sickle cell disease: natural history and management. Journal of Pediatrics 1985; 107 (2): 201-206

Contributor: Nick Shenker and Clare Wotton, April 2000
Reviewer: Mona Nabulsi

_{Clinical Question.}

_Patient	_{sickle cell disease}
_{Intervention or Exposure}	_prevalence
_Outcome	_{acute splenic sequestration}