Splenectomy versus conservative management for acute sequestration crises in people with sickle cell disease

  • Review
  • Intervention

Authors


Abstract

Background

Acute splenic sequestration crises are a complication of sickle cell disease, with high mortality rates and frequent recurrence in survivors of first attacks. Splenectomy and blood transfusion, with their consequences, are the mainstay of long-term management used in different parts of the world.

Objectives

To assess whether splenectomy (total or partial), to prevent acute splenic sequestration crises in people with sickle cell disease, improved survival and decreased morbidity in people with sickle cell disease, as compared with regular blood transfusions.

Search methods

We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register, which comprises of references identified from comprehensive electronic database searches and handsearching relevant journals and abstract books of conference proceedings.

Additional trials were sought from the reference lists of the trials and reviews identified by the search strategy.

Date of the most recent search: 27 August 2010.

Selection criteria

All randomized or quasi-randomized controlled trials comparing splenectomy (total or partial) to prevent recurrence of acute splenic sequestration crises with no treatment or blood transfusions in people with sickle cell disease.

Data collection and analysis

No trials of splenectomy for acute splenic sequestration were found.

Main results

No trials of splenectomy for acute splenic sequestration were found.

Authors' conclusions

Splenectomy, if full, will prevent further sequestration and if partial, may reduce the recurrence of acute splenic sequestration crises. However, there is a lack of evidence from trials showing that splenectomy improves survival and decreases morbidity in people with sickle cell disease. There is a need for a well-designed, adequately-powered, randomized controlled trial to assess the benefits and risks of splenectomy compared to transfusion programmes, as a means of improving survival and decreasing mortality from acute splenic sequestration in people with sickle cell disease.

Plain language summary

Removing spleens from people with sickle cell disease after a splenic sequestration compared to treating them with blood transfusions to prevent further attacks

In some people with sickle cell disease, red blood cells become trapped and destroyed in the spleen. This damages the spleen, which may become enlarged leading to splenic sequestration crises. These crises consist of abdominal pain, rapid heart rate and other symptoms. Such an attack can be fatal without prompt treatment. All or part of the spleen (splenectomy) is often removed after a person has survived such a crisis to try and prevent another one. This surgery may leave the individual at a higher risk of infection. We looked for trials which compared surgery to blood transfusions. We found no trials to provide reliable evidence about the risks or benefits of splenectomy for people with sickle cell disease after splenic sequestration. There is a need for a trial to assess the benefits and risks of splenectomy compared to transfusion programmes.