Increased red blood cell destruction, or haemolysis, occurs under two circumstances: when there is an abnormality of the red cells, as in sickle cell anaemia, thalassaemia or hereditary spherocytosis, or when a destructive process is at work, such as infection or autoimmune conditions. Fragile red cells are destroyed by the spleen, which becomes enlarged (Fig. below). Sickle-cell anaemia is most prevalent in the black American and African populations. Progression of the disease leads to repeated infarcts in various organs, including the spleen, which may eventually become shrunken and fibrosed. Patients have (non-obstructive) jaundice because the increased destruction of red blood cells (RBCs) releases excessive amounts of bilirubin into the blood.
Ultrasound images of Splenomegaly in hereditary spherocytosis.
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