Background: Sickle cell disease is an inherited autosomal recessive blood condition and is one of the most prevalent genetic blood diseases worldwide. Acute chest syndrome is a frequent complication of sickle cell disease, as well as a major cause of morbidity and the greatest single cause of mortality in children with sickle cell disease. Standard treatment may include intravenous hydration, oxygen as treatment for hypoxia, antibiotics to treat the infectious cause and blood transfusions may be given. This is an update of a Cochrane Review first published in 2010 and updated in 2016.
Objectives: To assess the effectiveness of blood transfusions, simple and exchange, for treating acute chest syndrome by comparing improvement in symptoms and clinical outcomes against standard care.
Search methods: We searched The Cochrane Cystic Fibrosis and Genetic Disorders Group's Haemoglobinopathies Trials Register, which comprises references identified from comprehensive electronic database searches and handsearching of relevant journals and abstract books of conference proceedings. Date of the most recent search: 30 May 2019.
Selection criteria: Randomised controlled trials and quasi-randomised controlled trials comparing either simple or exchange transfusion versus standard care (no transfusion) in people with sickle cell disease suffering from acute chest syndrome.
Data collection and analysis: Both authors independently selected trials and assessed the risk of bias, no data could be extracted.
Main results: One trial was eligible for inclusion in the review. While in the multicentre trial 237 people were enrolled (169 SCC, 42 SC, 15 Sβ⁰-thalassaemia, 11Sβ+-thalassaemia); the majority were recruited to an observational arm and only ten participants met the inclusion criteria for randomisation. Of these, four were randomised to the transfusion arm and received a single transfusion of 7 to 13 mL/kg packed red blood cells, and six were randomised to standard care. None of the four participants who received packed red blood cells developed acute chest syndrome, while 33% (two participants) developed acute chest syndrome in standard care arm. No data for any pre-defined outcomes were available.
Authors' conclusions: We found only one very small randomised controlled trial; this is not enough to make any reliable conclusion to support the use of blood transfusion. Whilst there appears to be some indication that chronic blood transfusion may play a roll in reducing the incidence of acute chest syndrome in people with sickle cell disease and albeit offering transfusions may be a widely accepted clinical practice, there is currently no reliable evidence to support or refute the perceived benefits of these as treatment options; very limited information about any of the potential harms associated with these interventions or indeed guidance that can be used to aid clinical decision making. Clinicians should therefore base any treatment decisions on a combination of; their clinical experience, individual circumstances and the unique characteristics and preferences of adequately informed people with sickle cell disease who are suffering with acute chest syndrome. This review highlights the need of further high quality research to provide reliable evidence for the effectiveness of these interventions for the relief of the symptoms of acute chest syndrome in people with sickle cell disease.
Copyright © 2020 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
Conflict of interest statement
There are no financial conflicts of interest and the review authors declare that they do not have any associations with any parties who may have vested interests in the results of this review.
Blood Transfusions for Treating Acute Chest Syndrome in People With Sickle Cell DiseaseS Dastgiri et al. Cochrane Database Syst Rev (8), CD007843. PMID 27574910. - ReviewWe found only one very small randomised controlled trial; this is not enough to make any reliable conclusion to support the use of blood transfusion. Whilst there appears …
Blood Transfusions for Treating Acute Chest Syndrome in People With Sickle Cell DiseaseD Alhashimi et al. Cochrane Database Syst Rev (1), CD007843. PMID 20091653. - ReviewThere is currently no reliable evidence to support or refute the effectiveness of blood transfusions as treatment options for acute chest syndrome in people with sickle c …
Preoperative Blood Transfusions for Sickle Cell DiseaseLJ Estcourt et al. Cochrane Database Syst Rev 4, CD003149. PMID 27049331. - ReviewThere is insufficient evidence from randomised trials to determine whether conservative preoperative blood transfusion is as effective as aggressive preoperative blood tr …
Blood Transfusion for Preventing Primary and Secondary Stroke in People With Sickle Cell DiseaseLJ Estcourt et al. Cochrane Database Syst Rev 1 (1), CD003146. PMID 28094851. - ReviewThere is no evidence for managing adults, or children who do not have HbSS sickle cell disease.In children who are at higher risk of stroke and have not had previous long …
Inhaled Nitric Oxide for Acute Chest Syndrome in People With Sickle Cell DiseaseA Al Hajeri et al. Cochrane Database Syst Rev (1), CD006957. PMID 18254121. - ReviewThere is a need for well-designed, adequately-powered randomised controlled trial to assess the benefits and risks of this form of treatment as an adjunct to established …