Course of the disease in hereditary spherocytosis

Removal of the spleen (splenectomy) results in striking and usually permanent improvement both in the symptoms and in the anaemia, and is carried out when the anaemia causes persistent impairment of health, when severe haemolytic crises have occurred, when other members of the family have died of the disease, or where there is evidence of gall bladder disease.

Hereditary Haemoglobinopathies (causing abnormal haemoglobin synthesis).

i.e. includes a wide range of blood disorders which in some cases results in the consequent haemolytic anaemia to be so severe, that fatal results occur. In other patients the disease may be so mild as to cause little or no inconvenience. Examples of hereditary haemoglobinopathies include Thalassaemia and Sickle Cell Disease.

There is no specific treatment, and splenectomy is contraindicated. The increase in immigration into the UK over the years of racial groups in whom haemoglobinopathies are common, has resulted in identification of considerable numbers of persons suffering from disease related to haemoglobinopathies. Very occasionally, refractory iron deficiency anaemia in British subjects without known Mediterranean ancestry may be due to Thalassaemia minor.