Haemolytic anaemias Flashcards
What is haemolytic anaemia?
Anaemia due to shorted RBC survival
What is incompletely compensated haemolysis?
RBC production unable to keep up with decreased RBC life span –> decreased Hb
What are the clinical findings of haemolytic anaemias?
- Jaundice
- Pallor
- Fatigue
- Gallstones - pigment
- Leg ulcers
- Folate deficiency
What is the peripheral blood film for Haemolytic anaemia?
Polychromatophilia (enlarged)
Nucleated RBC - (dark part within the cell)
Thrombocytosis
Neutrophilia with left shift
Schistocytes - fragmented and triangular RBC
Sickle cells
What are the bone marrow findings for Haemolytic anaemia?
Erythroid hyperplasia of bone marrow
Reticulocytosis - immature red blood cells increase due to an increased need for RBC production
How can you classify haemolytic anaemia?
- Inheritance
- Hereditary - hereditary spherocytosis
- Acquired - IHA
- Site of RBC destruction
- Intravascular - haemolytic transfusion
- Extravascular - autoimmune haemolysis
- Origin of RBC damage
- Intrinsic - G6PD deficiency
- Extrinsic - infections
What happens at the site of RBC destruction in a normal and abnormal situation?
What is hereditary spherocytosis?
Membrane disorder - mutations in the RBC membrane proteins
- Cells are sphere shape and are more fragile
- Increased risk of haemolysis - rupturing of RBC and released into surroundings
- Deeply stained on blood film
Findings:
- Neonatal jaundice
- Positive family history
- Pigment gallstones
Management:
- Monitor
- Folic acid
- Transfusion
- Splenectomy
What is G6P dehydrogenase deficiency?
Oxidation of Hb by oxidant radicals - resulting in denatured Hb which agregates and forms bodies which bind to membrane
Oxidised membrane proteins - reduced RBC deformability
Blood film - bite cells, blister cells and ghost cells
What is pyruvate kinase deficiency?
Deficiency will affect the energy generated in glycolytic pathway which is to maintain red cell shape and deformability
Causes chronic non-spherocytic haemolytic anaemia
What is a globin disorder?
Thalassaemia’s:
- Defect in the rate of synthesis alpha or beta globin chain - imbalance
- Excess unpaired globin chains are unstable
- Ineffective erythropoiesis
- B-thalassemia major - severe anemia, bone marrow expansion, iron overload and mild jaundice - nucleated RBC and tear drop cells
- Thalassaemia intermedia - increased bilirubin level
- Thalassaemia minor - confused with Fe deficiency
- Alpha-thalassaemia - splenomegaly
What is sickle cell disease?
All diseases as a result of inherited HbS caused by single nucleotide substitution
- HbSS is sickle cell anaemia
- HbAS - sickle cell trait
Findings:
- Chest syndrome, stroke
- Renal failure
- Avascular necrosis bone
- Anaemia
- Reticulocytotic
- Raised bilirubin
- Low creatinine
