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Haemolytic anaemia
 

Haemolytic anaemia

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This is a successful attempt of group of Ruhuna Medicos have done for their term seminar.

This is a successful attempt of group of Ruhuna Medicos have done for their term seminar.

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    Haemolytic anaemia Haemolytic anaemia Presentation Transcript

    • HaemolyticAnaemia
    • Definition -
      A disorder in which the red blood cells are destroyed Prematurely.
      Cells are broken down at a faster rate than the bone marrow produce new cells
    • What is haemolysis?
      The destruction or dissolution of red Blood cells which relaeseHaemoglobin
      Also known as ERYTHROCYTOLYSIS ,ERYTHROLYSIS
      Extravascular
      Intravascular
    • Classification
      Aquired
      Inherited
      Red cell membrane defect
      Haemoglobin Abnormalities
      Metabolic Defects
      Immune
      2. Non Immune
    • 1. Red Cell Membrane defects
      Eg:-
      Hereditary Spherocytosis
      Hereditary Elliptocytosis
    • Hereditary Spherocytosis
      Inherited as Autosomal Dominant
      Present in Approximately 1:5000
      Defects in Red Cell Membrane
      • Most Characterized defect is in defficiency of- SPECTRIN
      • Most Common- ANQUIRUM
    • HeredetarySpherocytosis
      Spherical rather than disk shaped
      Cells more Rigid and less deformable
      Unable to pass through Splenic micro circulation
      Anaemia
      Cell Lysis
    • Hereditary Elliptocytosis
      Aauatosmal Dominant
      Red Cells are Elliptical
      Due to alterations in Structure or Quantity of Cytoskeleton Proteins
      Eg- ,βSpectrin
    • Hereditary Stomaocytosis
      • Red cells in which the pale central area appears
      • Due to Asymmetric increased passing of Na+ and K+ Ions in RBC
      (Passive leak is Increased)
      • Laeding to Haemolysis
    • 2. Haemoglobin Abnormalities
      Abnormalities Occur in
      Globin Chain Production
      Structure of the Globin Chain
      SICKLE CELL ANAEMIA
      THALASSAEMIA
    • Thalassaemia
      An inherited autosomal recessive haemolytic disease
      • Imbalance occur in the Synthesis of Globin Chains
      • Precipitation of Globin Chains in mature Red Cells
      • Haemolysis
    • Thalassaemia
      Thalassaemia
      βThalassaemia
      • Caused By Point Mutation
      • Caused By gene Deletion
      • Defects in synthesis of Globin Chain
      Thalassaemia Minor{Trait}
      Thalassaemia Major
      {CooleysAnaemia}
      ThalassaemicIntermedia
    • Thalassaemia Minor{Trait}
      Heterozygous carrer state
      Asymptomatic
      Anaemia Mild or absent
      Red Cells are Hypochromic and Microcytic
    • Thalassaemia Major{CooleysAnaemia}
      Severe Anaemia From 3-6 Months
      Haepatosplenomegally and Bone expansion
      Require Regular Transfusion
    • ThalassaemiCIntermedia
      Symptomatic with Moderate Anaemia
      Rarely required Transfusion
    • Sickle Cell Anaemia
      An autosomal Recessive Type of disease
      Due to Point Mutaion(Partially acceptable)
      2nd Base is Changed
      *The Sixth Amino Acid Residue
      Valaine
      (Hydrophobic)
      Glutamate
      (Hydrophilic)
    • Abnormal Type of HaemoglobinHbS
      Containing Faulty Beta Chain in the Hb Membrane
      Hb is exposed to Low Oxygen concentrations
      Precipitates into long Crystals in RBC
      Sickle Appearance
      Highly Fragile
      Causing Anaemia
    • 3. Metabolic Defects
      Glucose 6 PhosphateDehydrogenase(G6PD)Defficiency
      *Important in the HexoseMonophosphate shunt
      Oxidizing
      Glucose -6-Phosphate 6-Phosphoglycerte
      NADP NADPH
      This reaction is necessary in Red Cells
      It is the only source of NADPH
      2. Used via Glutathione to Protect the Red Cell from Oxidative damage.
    • PyruvateKinasedefficiency
      Inherited as Autosamal Recessive
      Reduced Production of ATP
      • Cause Rigid Red Cells
      PyruvateKinase
      Important in the Glycolysis
      PhosphoenolpruvatePyruvate
      ADP ATP
    • AquiredHaemolyticAnaemia
      1.) Immune
      Auto Immune HaemolyticAnaemia
      -autoantibodies against erythrocytes
      Drug Induced Immune HaemolyticAnaemia
      -Interaction between drug & RBC membrane produce antibodies
      AlloimmuneHaemolyticAnaemia
      - Haemolytic Disease in New Born
      - Haemolytic Transfusion Reaction
      - After Transplantation(BM,Renal or cardiac)
    • 2.Non Immune
      Paroxysmal Nocturnal Haemoglobnuria (PNH) -Rare disease
      -Intravascular Haemolysis Occurs
      Mechanical Haemolytic Anaemia
      -Red Cells may injured by,
      -Physical Trauma in the Circulation.
    • The end