Haemolytic anaemia

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

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Haemolytic anaemia

  1. 1. HaemolyticAnaemia<br />
  2. 2. Definition -<br />A disorder in which the red blood cells are destroyed Prematurely.<br />Cells are broken down at a faster rate than the bone marrow produce new cells<br />
  3. 3. What is haemolysis?<br />The destruction or dissolution of red Blood cells which relaeseHaemoglobin<br />Also known as ERYTHROCYTOLYSIS ,ERYTHROLYSIS<br />Extravascular<br />Intravascular<br />
  4. 4. Classification<br />Aquired<br />Inherited<br />Red cell membrane defect<br />Haemoglobin Abnormalities<br />Metabolic Defects<br />Immune<br />2. Non Immune<br />
  5. 5. 1. Red Cell Membrane defects<br />Eg:-<br />Hereditary Spherocytosis<br />Hereditary Elliptocytosis<br />
  6. 6. Hereditary Spherocytosis<br />Inherited as Autosomal Dominant <br />Present in Approximately 1:5000<br />Defects in Red Cell Membrane<br /><ul><li>Most Characterized defect is in defficiency of- SPECTRIN
  7. 7. Most Common- ANQUIRUM</li></li></ul><li>HeredetarySpherocytosis<br />Spherical rather than disk shaped<br />Cells more Rigid and less deformable<br />Unable to pass through Splenic micro circulation<br />Anaemia<br />Cell Lysis<br />
  8. 8. Hereditary Elliptocytosis<br />Aauatosmal Dominant<br />Red Cells are Elliptical<br />Due to alterations in Structure or Quantity of Cytoskeleton Proteins<br />Eg- ,βSpectrin<br />
  9. 9. Hereditary Stomaocytosis<br /><ul><li>Red cells in which the pale central area appears
  10. 10. Due to Asymmetric increased passing of Na+ and K+ Ions in RBC</li></ul> (Passive leak is Increased)<br /><ul><li>Laeding to Haemolysis</li></li></ul><li>2. Haemoglobin Abnormalities<br />Abnormalities Occur in<br />Globin Chain Production<br />Structure of the Globin Chain<br />SICKLE CELL ANAEMIA<br />THALASSAEMIA<br />
  11. 11. Thalassaemia<br />An inherited autosomal recessive haemolytic disease<br /><ul><li>Imbalance occur in the Synthesis of Globin Chains
  12. 12. Precipitation of Globin Chains in mature Red Cells
  13. 13. Haemolysis</li></li></ul><li>Thalassaemia<br />Thalassaemia<br />βThalassaemia<br /><ul><li>Caused By Point Mutation
  14. 14. Caused By gene Deletion
  15. 15. Defects in synthesis of Globin Chain</li></ul>Thalassaemia Minor{Trait}<br />Thalassaemia Major<br />{CooleysAnaemia}<br />ThalassaemicIntermedia<br />
  16. 16. Thalassaemia Minor{Trait}<br />Heterozygous carrer state<br />Asymptomatic<br />Anaemia Mild or absent<br />Red Cells are Hypochromic and Microcytic<br />
  17. 17. Thalassaemia Major{CooleysAnaemia}<br />Severe Anaemia From 3-6 Months<br />Haepatosplenomegally and Bone expansion<br />Require Regular Transfusion<br />
  18. 18. ThalassaemiCIntermedia<br />Symptomatic with Moderate Anaemia<br />Rarely required Transfusion<br />
  19. 19. Sickle Cell Anaemia<br />An autosomal Recessive Type of disease<br />Due to Point Mutaion(Partially acceptable)<br />2nd Base is Changed<br />*The Sixth Amino Acid Residue<br />Valaine<br />(Hydrophobic)<br />Glutamate<br />(Hydrophilic)<br />
  20. 20. Abnormal Type of HaemoglobinHbS<br />Containing Faulty Beta Chain in the Hb Membrane<br />Hb is exposed to Low Oxygen concentrations<br />Precipitates into long Crystals in RBC<br />Sickle Appearance<br />Highly Fragile<br />Causing Anaemia<br />
  21. 21. 3. Metabolic Defects<br />Glucose 6 PhosphateDehydrogenase(G6PD)Defficiency<br />*Important in the HexoseMonophosphate shunt<br />Oxidizing<br /> Glucose -6-Phosphate 6-Phosphoglycerte<br />NADP NADPH <br />This reaction is necessary in Red Cells<br />It is the only source of NADPH<br />2. Used via Glutathione to Protect the Red Cell from Oxidative damage.<br />
  22. 22. PyruvateKinasedefficiency<br />Inherited as Autosamal Recessive<br />Reduced Production of ATP<br /><ul><li>Cause Rigid Red Cells</li></ul>PyruvateKinase<br />Important in the Glycolysis<br />PhosphoenolpruvatePyruvate<br />ADP ATP<br />
  23. 23. AquiredHaemolyticAnaemia<br />1.) Immune<br />Auto Immune HaemolyticAnaemia<br />-autoantibodies against erythrocytes<br />Drug Induced Immune HaemolyticAnaemia<br />-Interaction between drug & RBC membrane produce antibodies<br />AlloimmuneHaemolyticAnaemia<br />- Haemolytic Disease in New Born<br />- Haemolytic Transfusion Reaction<br />- After Transplantation(BM,Renal or cardiac)<br />
  24. 24. 2.Non Immune<br />Paroxysmal Nocturnal Haemoglobnuria (PNH) -Rare disease<br /> -Intravascular Haemolysis Occurs<br />Mechanical Haemolytic Anaemia<br /> -Red Cells may injured by, <br /> -Physical Trauma in the Circulation.<br />
  25. 25. The end<br />

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