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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
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 relaese HaemoglobinAlso known as ERYTHROCYTOLYSIS ,ERYTHROLYSIS
ExtravascularIntravascular
CLASSIFICATION
Inherited Aquired
1. Red cell membrane
defect
2. Haemoglobin
Abnormalities
3. Metabolic Defects
1. 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
HEREDETARY SPHEROCYTOSIS
Spherical rather than disk shaped
Cells more Rigid and less deformable
Unable to pass through Splenic micro circulation
Cell LysisAnaemi
a
HEREDITARY ELLIPTOCYTOSIS
Aauatosmal Dominant
Red Cells are Elliptical
Due to alterations in
Structure or Quantity of
Cytoskeleton Proteins
Eg- , β Spectrin
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
Abnormalities Occur in
Globin Chain Production
Structure of the Globin Chain
THALASSAEMIA SICKLE CELL ANAEMIA
2. Haemoglobin Abnormalities
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 β Thalassaemia
•Caused By gene Deletion
•Defects in synthesis of
Globin Chain
Thalassaemia Minor{Trait}
Thalassaemic Intermedia
Thalassaemia Major{Cooleys Anaemia}
• Caused By Point Mutation
THALASSAEMIA MINOR{TRAIT}
Heterozygous carrer state
Asymptomatic
Anaemia Mild or absent
Red Cells are Hypochromic and Microcytic
THALASSAEMIA MAJOR{COOLEYS ANAEMIA}
Severe Anaemia From 3-6 Months
Haepatosplenomegally and Bone expansion
Require Regular Transfusion
THALASSAEMIC INTERMEDIA
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
Glutamate(Hydrophilic)
Valaine(Hydrophobic)
*The Sixth Amino Acid Residue
Abnormal Type of Haemoglobin HbS
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
Glucose 6 PhosphateDehydrogenase(G6PD)Defficiency
*Important in the Hexose Monophosphate shuntOxidizing Glucose -6-Phosphate 6-Phosphoglycerte
NADP NADPH
This reaction is necessary in Red Cells1. It is the only source of NADPH2. Used via Glutathione to Protect the Red Cell from Oxidative damage.
3. Metabolic Defects
Inherited as Autosamal Recessive Reduced Production of ATP
Phosphoenolpruvate Pyruvate
Pyruvate Kinase
ADP ATP
•Cause Rigid Red Cells
Important in the Glycolysis
Pyruvate Kinase defficiency
AQUIRED HAEMOLYTIC ANAEMIA
1.) Immune
Auto Immune Haemolytic Anaemia
Drug Induced Immune Haemolytic Anaemia
Alloimmune Haemolytic Anaemia
-autoantibodies against erythrocytes
-Interaction between drug & RBC membrane produce antibodies
- Haemolytic Disease in New Born- Haemolytic Transfusion Reaction- After Transplantation(BM,Renal or cardiac)
Mechanical Haemolytic Anaemia-Red Cells may injured by, -Physical Trauma in the Circulation.
Paroxysmal Nocturnal Haemoglobnuria (PNH)
-Rare disease-Intravascular Haemolysis Occurs
2. Non Immune
THE END