Haemolytic Anaemia Flashcards

1
Q

Definition

A

Premature erythrocyte breakdown causing shortened erythrocyte life span (< 120 days) with anaemia

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2
Q

Aetiology (hereditary)

A

o Membrane Defects
• Hereditary spherocytosis
• Elliptocytosis

o Metabolic Defects
• G6PD deficiency
• Pyruvate kinase deficiency

o Haemoglobinopathies
• Sickle cell disease
• Thalassemia

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3
Q

Aetiology (acquired)

A

o Autoimmune
• Antibodies attach to erythrocytes causing intravascular and extravascular
haemolysis

o Isoimmune
• Transfusion reaction
• Haemolytic disease of the newborn

o Drugs
• Penicillin
• Quinine
• NOTE: this is caused by the formation of a drug-antibody-erythrocyte complex

o Trauma
• Microangiopathic haemolytic anaemia (caused by RBC fragmentation in abnormal microcirculation)
▪ E.g. haemolytic uraemic syndrome, DIC, malignant hypertension

o Infection
• Malaria
• Sepsis

o Paroxysmal nocturnal haemoglobinuria

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4
Q

Epidemiology

A
  • COMMON
  • Genetic causes are prevalent if African, Mediterranean and Middle Eastern populations
  • Hereditary spherocytosis is the most common inherited haemolytic anaemia in northern Europe
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5
Q

Presenting symptoms

A
  • Jaundice
  • Haematuria
  • Anaemia
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6
Q

Signs on physical examination

A
  • Pallor
  • Jaundice
  • Hepatosplenomegaly
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7
Q

Investigations (bloods)

A
o FBC:
• Low Hb
• High reticulocytes
• High MCV
• High unconjugated bilirubin
• Low haptoglobin (a protein that binds to free Hb released by red blood cells)

o U&Es

o Folate

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8
Q

Investigations (blood film)

A

o Leucoerythroblastic picture
o Macrocytosis
o Nucleated erythrocytes or reticulocytes
o Polychromasia

o May identify specific abnormal cells such as:
• Spherocytes
• Elliptocytes
• Sickle cells
• Schistocytes
• Malarial parasites
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9
Q

Investigations (urine)

A

o High urobilinogen
o Haemoglobinuria
o Haemosiderinuria

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10
Q

Investigations (other)

A

• Direct antiglobulin test/Coombs’ Test
o Tests for autoimmune haemolytic anaemia
o Identifies erythrocytes coated with antibodies

• Osmotic fragility test or Spectrin mutation analysis
o Identifies membrane abnormalities

• Ham’s Test
o Lysis of erythrocytes in acidified serum in paroxysmal nocturnal haemoglobinuria

• Hb Electrophoresis or Enzyme Assays
o To exclude other causes

• Bone Marrow Biopsy (rarely performed)

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