By ULY CLINIC

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Hereditary Haemolytic Anaemia (HHA)

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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.

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HHA defect may be in

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Membrane  defects           

• Hereditary spherocytosis           

• Hereditary elliptocytosis

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Metabolism  disorders        

• G6PD deficiency             

• Pyruvate kinase deficiency

 

Haemoglobin defects

• Abnormal haemoglobin such as Hb S, C

• Unstable Hb

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Clinical Features:

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• 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  

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General Treatment:

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1. Remove the underlying cause

2. Blood transfusion if anaemia is severe 

3. Plasmapheresis

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

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Pharmacological Management

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Immunosuppressant’s  

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

 

AND

• Cyclosphophamide 60mg/m2 IV

 

OR

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

 

OR

• Cyclosporin 2–5mg/

 

OR 

• 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

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Updated on, 06.11.2020

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References

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1. STG

2. Boston childrens hospital. Hemolytic anemia. https://www.childrenshospital.org/conditions-and-treatments/conditions/h/hemolytic-anemia#. Accessed 06.11.2020