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Haemoglobinopathies 

Haemoglobinopathies
Chapter:
Haemoglobinopathies
Author(s):

Paul L.F. Giangrande

DOI:
10.1093/med/9780199550647.003.0005
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date: 20 February 2018

Haemoglobinopathies are commonly inherited disorders of haemoglobin synthesis

Thalassaemia is commonest around the Mediterranean countries and has skeletal manifestations due to massive marrow expansion with thinning of the cortex

Sickle cell crises occur in homozygotes and are a result of venous occlusion causing avascular necrosis. Infection and exposure to cold can sometimes precipitate these painful events

If surgery is needed blood cross-match must be carefully performed in advance, the theatre should be kept warm, and the hydration and acid/base balance of the patient monitored carefully.

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