source: British Journal of Haematology

year: 2015

authors: Jo Howard, Nicholas Hart, Marilyn Roberts-Harewood, Michelle Cummins, Moji Awogbade, Bernard Davis

Sickle cell disease (SCD) affects 12 000–15 000 individuals in the UK. Whilst homozygous SCD (sickle cell anaemia — HbSS) is the most common and severe genotype, and is where most of the evidence exists, this guidance should be used for all genotypes of SCD.

Acute chest syndrome (ACS) is defined as an acute illness characterized by fever and/or respiratory symptoms, accompanied by a new pulmonary infiltrate on chest X-ray (Charache et al, 1979; Ballas et al, 2010). This definition encompasses cases both where an infective organism is isolated and where no infective cause is identified. It is unique to SCD but in some cases ACS may appear to be similar to bacterial pneumonia in a patient without SCD. ACS may have a severe clinical course and can progress rapidly from mild hypoxia to respiratory failure and death. The presence of hypoxia is not included in the definition, but in clinical practice, hypoxia is a useful predictor of severity and outcome (Vichinsky et al, 1997, 2000).

Historically, ACS is one of the most common causes of death in patients with SCD (Platt et al, 1994; Lucas et al, 2008), although mortality is improving with improved medical management (Fitzhugh et al, 2010). ACS can also be associated with significant morbidity, including long-term parenchymal lung damage, pulmonary vascular abnormality and neurological sequelae. Patients may present to hospital acutely unwell with ACS or ACS may develop during a hospital admission following a painful crisis or post-operatively.

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