Chronic features

Patients with sickle cell disease are chronically anaemic (7-8 g/dl) with a hyperdynamic circulation. Splenomegaly is common in youth but, with progressive episodes of infarction, splenic atrophy occurs leading to an increased risk of infection, particularly pneumococcal.

Chronic features include skin ulcers, renal failure, avascular bone necrosis (± supervening osteomyelitis, especially Salmonella), hepatomegaly, jaundice and cardiomyopathy. Sudden cardiac death is not uncommon, usually before the age of 30.

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