Sickle cell crises
Sickle cell crises occur most commonly in haemoglobin S homozygotes. Patients with mixed haemoglobinopathies may have sickling episodes e.g. SC disease.
Sickle cell crises do not occur in Hb S heterozygotes.
Most common sites of pain onset are the back or trunk, and pain may then radiate to the limbs (1):
- duration is usually a few hours to days, but in some cases, it may persist for weeks
- consider the complication of acute chest syndrome* in patients with fever and respiratory signs and symptom
* acute chest syndrome
- a combination of pulmonary infarction, infection, and fat embolism
- caused by sickling and ischaemia in deep tissues of the chest wall
The various sickle cell crises and their acute management are described below.
Reference
- Charles K S, Friday M, Rochford E. Acute painful crisis in adults with sickle cell disease BMJ 2024; 386 :e075099 doi:10.1136/bmj-2023-075099
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