Haemolytic anaemias2 3.57.51 PM

Haemolytic anaemias2 3.57.51 PM - haemolyticanaemias...

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Inherited and acquired  haemolytic anaemias Dr. Suhair Abbas Ahmed
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Hereditary haemolytic anaemias Membrane defects  – congenital  spherocytosis Metabolic defects  – G6PD enzyme  deficiency Haemoglobin defects Qualitative defects – sickle cell anaemia Quantitative defects – Thalassaemia 
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Membrane defects Hereditary spherocytosis South East Asian ovalocytosis
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Hereditary spherocytosis (HS) It is the most common hereditary  haemolytic anaemia in  North  Europeans .
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Pathogenesis of HS It is caused by a  defect  in the  proteins   involved in the interactions between the  membrane cytoskeleton and the lipid  bilayer of the red cell. (  ankyrin spectrin   and  pallidin ).
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Clinical features The inheritance is  autosomal   dominant. Rarely it may be autosomal recessive. The  anaemia  may present at any age  from infancy to old age. Jaundice  is fluctuating. Splenomegaly  occurs in most of the  patients. Pigment  gall stones  are frequent.
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Haematological findings in HS Anaemia  is usual. Reticulocytosis  5-20% Microsherocytes  are seen in the blood  film. (densely staining with smaller  diameters than normal red cells).
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Reticulocytosis is a  feature of increased  red cell production. New methylene blue
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This note was uploaded on 12/24/2011 for the course STEP 1 taught by Professor Dr.aslam during the Fall '11 term at Montgomery College.

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Haemolytic anaemias2 3.57.51 PM - haemolyticanaemias...

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