Main » Infectious diseases » Miscellaneous Disorders » Genetic blood dyscrasias
The most important abnormal haemoglobins in the tropics are Hb S C. E. Thalassaemia which is a failure of foetal haemoglobin synthesis is also widespread. Hb D has a limited distribution and is clinically mild. The abnormal haemoglobins also occur in Central and South America and among blacks in the United States.
Dictribution map of haemoglobinopathies.
Sickle cell disease
Photo 1. Dactylitis due to sickle cell disease. Severe bilateral dactylitis is a common presentation of sickle cell disease in children.
Photo 2. X-ray of hands in sickle cell disease. Note destructive changes in small bones, the result of multiple infarction complicated by infection (in this case by a Salmonella species).
Photo 3. Bossing of scull. Bossing of the skull due to hyperplasia of the marrow is another feature of sickle cell disease. Similar appearances may be seen in thalassaemia and in any other severe congenital haemolytic anaemia.
Photo 4. X-ray of scull in thalassaemia. Haemoglobin E disease produces this typical 'hair-on-end' appearance of the skull in X-rays.
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