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

1. HaemolyticAnaemia

2. 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

3. What is haemolysis? The destruction or dissolution of red Blood cells which relaeseHaemoglobin Also known as ERYTHROCYTOLYSIS ,ERYTHROLYSIS Extravascular Intravascular

4. Classification Aquired Inherited Red cell membrane defect Haemoglobin Abnormalities Metabolic Defects Immune 2. Non Immune

5. 1. Red Cell Membrane defects Eg:- Hereditary Spherocytosis Hereditary Elliptocytosis

8. Hereditary Elliptocytosis Aauatosmal Dominant Red Cells are Elliptical Due to alterations in Structure or Quantity of Cytoskeleton Proteins Eg- ,βSpectrin

12. Precipitation of Globin Chains in mature Red Cells

14. Caused By gene Deletion

15. Defects in synthesis of Globin ChainThalassaemia Minor{Trait} Thalassaemia Major {CooleysAnaemia} ThalassaemicIntermedia

16. Thalassaemia Minor{Trait} Heterozygous carrer state Asymptomatic Anaemia Mild or absent Red Cells are Hypochromic and Microcytic

17. Thalassaemia Major{CooleysAnaemia} Severe Anaemia From 3-6 Months Haepatosplenomegally and Bone expansion Require Regular Transfusion

18. ThalassaemiCIntermedia Symptomatic with Moderate Anaemia Rarely required Transfusion

19. 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)

20. 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

21. 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.

23. 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)

24. 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.

25. The end