Acute chest syndrome in sickle cell anaemia
Acute chest syndrome in sickle cell anaemia is a leading thoracic complication as well as leading cause of mortality in those affected by sickle cell disease 3.
For a general discussion of sickle cell disease, please refer to the article : sickle cell disease.
Patient's may present with acute fever, cough and wheezing on a background of established sickle cell disease.
The pathological basis is thought to be from pulmonary microvascular occlusion and haemolysis from distorted sickle cells as a result of a several factors including exposure to low O2 tension.
Typically seen as segmental / sub segmental atelectasis +/- consolidation with a lower lobe predilection +/- pleural effusion.
A chest radiograph may also show other sequelae from sickle cell disease such as bone infarcts, rib enlargement and cardiomegaly (from anaemia).
May show a mosaic perfusion pattern +/- a pleural effusion.
General imaging differential considerations include
- 1. Bhalla M, Abboud MR, Mcloud TC et-al. Acute chest syndrome in sickle cell disease: CT evidence of microvascular occlusion. Radiology. 1993;187 (1): 45-9. Radiology (abstract) - Pubmed citation
- 2. Lonergan GJ, Cline DB, Abbondanzo SL. Sickle cell anemia. Radiographics. 21 (4): 971-94. Radiographics (full text) - Pubmed citation
- 3. Neumayr L, Lennette E, Kelly D et-al. Mycoplasma disease and acute chest syndrome in sickle cell disease. Pediatrics. 2003;112 (1): 87-95. doi:10.1542/peds.112.1.87 - Pubmed citation
- 4. Maitre B, Habibi A, Roudot-thoraval F et-al. Acute chest syndrome in adults with sickle cell disease. Chest. 2000;117 (5): 1386-92. doi:10.1378/chest.117.5.1386 - Pubmed citation
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