Haematology >
Haemolytic Anaemia

“Anaemia secondary to reduced survival of red blood cells (RBCs)”

  • Destruction of RBCs- broadly defined as a reduction in the life span below 100 days (normal 110-120 days)
  • Classifications:
    • Inherited vs Acquired
    • Intravascular vs Extravascaular
Risk Factors
  • Family history
  • Genes for metabolic, haemoglobin, and membrane defects
  • Drugs- penicillins, cephalosporins, sulfonamides, sulfasalazine
  • Infections – mycoplasma pneumoniae
Aetiology
  • Genetic:
    • RBC membrane abnormalities- hereditary spherocytosis
    • Haemoglobin abnormalities- sickle cell, thalassaemia
    • Metabolic defects- G6PD deficiency
  • Acquired:
    • Immune- haemolytic disease of newborn, blood transfusion reaction
    • Autoimmune- idiopathic, SLE, chronic lymphatic leukaemia, infections
    • Drugs
Pathophysiology
  • Two mechanisms:
    • Intravascular
      • Due to complement fixation, trauma, or other extrinsic factors
      • Prosthetic valves, cardiac valves, G6PD deficiency, disseminated intravascular coagulation
    • Extravascular (most common)
      • RBCs removed from the circulation because they are either intrinsically defective or because of bound immunoglobulins to their surfaces
Clinical Presentation
  • Symptoms:
    • Fatigue
    • Weakness
    • Paraesthesia
    • Dyspnoea
    • GI symptoms (nausea, dyspepsia)Weight loss
  • Signs:
    • Atrophic glossitis
    • Pallor
    • Fever
    • Splenomegaly
    • Evidence of underlying disease
  • Haemolysis:
    • Jaundice
    • Abdominal pain (eg gallstones)
    • Dark urine (haemoglobinuria secondary to intravascular haemolysis)
Investigations
  • Haemolytic screen:
    • FBC including reticulocyte count
    • Blood film
    • LDH
    • LFTs (bilirubin)
    • Serum haptoglobin
  • Other tests:
    • Direct anti-globulin test
    • Indirect anti-globulin test
    • Haemoglobin electrophoresis
    • Sickle cell screening
  • Traditionally causes a NORMOCYTIC ANAEMIA
Management
  • Conservative: n/a
  • Medical:
    • Administer folic acid
    • Discontinue meds causing haemolysis
    • Transfusion therapyIron therapy
    • Autoimmune haemolytic anaemia therapy:
      • Corticosteroids
      • Azathioprine and cyclophosphamide if steroids fail
      • Rituximab
  • Surgical:
    • Splenectomy
Complications
  • High-output cardiac failure
  • Jaundice
  • Iron deficiency

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