HAEMOLYTIC ANAEMIA

1. Haemolytic State & Haemolytic Anaemia(HA)

2. Haemolysis as minor component 3. Classification Of HA Intrinsic vs Extrinsic Intravascular vs Extravascular Inherited vs Acquired

Classification 1. Intrinsic Defects A. Inherited i. Membrane Defects a) Hereditary spherocytosis b) Hereditary elliptocytosis c) Hereditary pyropoikilocytosis d) Hereditary xerocytosis & hydrocytosis ii. Haemoglobin Defects a) Haemoglobinopathies b) Thalassemias c) Double heterozygous

iii. Enzymes Defects a. Impaired ATP synthesis-PK def, ADA overactivity, Pyrimidine 5 Nucleosidase def b. Glucose 6 Phosphate dehydrogenase def B. Acquired i. Membrane defect a. Paraoxysmal nocturnal haemoglobinuria

2. Extrinsic Defects A. Acquired i. Immune Mediated a. AIHA b. Haemolytic disease of newborn c. Mismatch Tx d. Immune drug induced haemolytic anaemia ii. Nonimmune a. Mechanical Cardiac March MAHA

iii. Miscellaneous
a. Drug b. Infection c. Burns d. Lead poisoning Investigation of HA ? Haemolytic ? Where is the haemolysis occurring Intravascuar/Extravascular ? Why is haemolysis occurring-aetiology

Is it Haemolysis?
1. Evidence of haemoglobin breakdown a. Bilirubinemia b. Faecal stercobilinogen c. Urinary urobilinogen d. plasma haptoglobin e. plasma haemopexin f. Methhaemalbuminemia g. Haemoglobinemia h. Haemoglobinuria i. Haemosiderinuria

Is it Haemolysis? 2. Evidence of RBC breakdown I a. Spherocytosis b. Fragmented RBCs c. Heinz bodies d. Increased osmotic fragility e. Raised LDH levels 3. Evidence of erythroid hyperplasia a. Polychromatophilia/ Reticulocytosis b. Hyperplastic marrow c. Normoblastemia d. Roentgenographic evidence e. Increased PIT

Site-Intravascular vs Extravascular

Feature
Bilirubin Haptoglobin Haemopexin Hbemia Hburia Haemosiderinuria

Intra
Raised Reduced Reduced Present Present Present

Extra
Raised Reduced Normal Absent Absent Absent

Methhaemalb

Present

Absent

Causes of Intravascular Haemolysis

1. Enzymopathies 2. CHAD 3. PCH 4. PNH 5. Mismatch blood tx-major 6. Burns 7. C welchii infection 8. Malaria & Blackwater fever 9. Snake bite 10.Mechanical HA 11.HUS 12.Toxaemia

Causes of Extravascular Haemolysis 1. 2. 3. 4. 5. Membrane defects Haemoglobin defects Warm Ab AIHA Mismatch Tx- alloantibody type Immune Drug associated HA

Cause of HA-Aetiology Special Tests Membrane electrophoresis Ultrastructural memb study Osmotic fragility Abnormal sentivity to heat Acidified serum test/ Sucrose lysis test Hb electophoresis / Hb variant analysis Column chromatography Alk denaturation test Enzyme screening & Variant analysis Sickling prep Hb-S solubility test Coombs test Ab specificity & thermal amplitude test