Haemoglobin SC (HbSC) disease accounts for 30% of cases of sickle cell disease in the United Kingdom and the United States. Unlike other sickle cell carriers, who are relatively asymptomatic, people with HbSC disease have a combination of genotypes with the potential to cause considerable morbidity due to intracellular water loss. Patients can present with acute pain, acute chest syndrome, proliferative retinopathy, splenic and renal complications, or stroke. We present a young man with HbSC disease who developed acute compartment syndrome. This is only the second report of this syndrome in a patient with HbSC disease. This is a very rare complication in HbSC disease, but it can have serious implications.

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