Haematology Made Simple : Hereditary Spherocytosis

Hereditary Spherocytosis is an autosomal dominant disorder.

It is characterized by lack of spectrin in the red cell membrane which cases the cells to become spheres instead of being normal,flexible and durable biconcave discs.

The poorly flexible spherical cells are thus unable to pass through small fenestrations in the splenic red pulp and hemolysis takes place when the red cells are trapped within the spleen.

The treatment for most patients include : supportive care with oral folic acid and blood transfusions during periods of severe anemia.

Splenectomy is considered if patients have modrate to severe spherocytosis or are refractory to medical treatment.

However splenectomy is associated with severe sepsis risk upto 30 years after the procedure with encapsulated bacteria,most common being Streptococcus pneumoniae although it will abolish the risk of life threatening anemia and  regular blood transfusons.

To avoid this risk of sepsis, anti pneumococcal,Haemophilus and meningococcal vaccines should be given several weeks before the procedure.