Sickle cell disease

 Clinical features :

1) Anaemia - not iron deficiency

2) Painful crises - Increased risk of infection

3) Acute chest syndrome - 

fever, tachypnoea, pleuritic chest pain, leucocytosis, pulmonary infiltration - Splenic sequestration

4) Retinopathy, leg ulcers, aseptic bone necrosis

5) Renal papillary necrosis, Stroke

- Features of the double heterozygote sickle cell beta-thalassaemia are indistinguishable from those of sickle cell disease.