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Hereditary Haemolytic Anaemia (HHA)

HHA sometime called inherited hemolytic anemia or intrinsic hemolytic anemia is caused by a defect in the red blood cells resulting from failing to function properly of one or more genes that control red blood cell production . With these conditions, red blood cells are destroyed earlier than normal.

HHA defect may be in

Membrane  defects           

  • Hereditary spherocytosis           

  • Hereditary elliptocytosis

Metabolism  disorders        

  • G6PD deficiency             

  • Pyruvate kinase deficiency


Haemoglobin defects

  • Abnormal haemoglobin such as Hb S, C

  • Unstable Hb

Clinical Features:

  • The disease may occur at any age and sex

  • Patient may present with symptom and features of Anaemia

  • Symptoms are usually slow in onset however rapidly developing anaemia can occur

  • Splenomegaly is common but no always observed

  • Jaundice  

General Treatment:

  1. Remove the underlying cause

  2. Blood transfusion if anaemia is severe 

  3. Plasmapheresis

Note: After supportive treatment refer to higher health facility with adequate expertise and facilities

Pharmacological Management


Prednisolone 1–1.5mg/kg/day (PO) for 1–3 weeks until Hb is greater   than 10g/dl 



  • Cyclosphophamide 60mg/m2 IV



  • Azathioprine 100–150mg/mg (PO) daily 



  • Cyclosporin 2–5mg/



  • High dose immunoglobulin 400mg/kg daily IV for 5 days 


Note: Folic acid is 5mg (PO) daily should be given to severe cases 


Surgical Management

  • Splenectomy may be considered in those who fail to respond

Updated on, 06.11.2020


  1. STG

  2. Boston childrens hospital. Hemolytic anemia. Accessed 06.11.2020

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