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By ULY CLINIC

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

Immunosuppressant’s  

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

Updated on, 06.11.2020

References

  1. STG

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

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