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ZERO TO FINALS > HAEMOLYTIC ANAEMIAS > Flashcards

HAEMOLYTIC ANAEMIAS Flashcards

1
Q

What are the causes of acquired haemolytic anaemia?

A
  1. Autoimmune haemolytic anaemia
  2. Alloimmune haemolytic anaemia (transfusions reactions
  3. Paroxysmal nocturnal haemoglobuinuria
  4. Microangiopathic haemolytic anaemia
  5. Prosthetic valve realted haemolysis
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2
Q

What are the causes of inherited haemolytic anaemias?

A
  1. Hereditary spherocytosis
  2. Hereditary elliptocytosis
  3. Thalassaemia
  4. Sickle cell anaemia
  5. G6PD
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3
Q

What features are seen in haemolytic anaemia?

A
  • anaemia due to the reduction in circulating red blood cells
  • splenomegaly as the spleen becomes filled with destroyed red blood cells
  • jaundice as bilirubin is released during the destruction of red blood cells
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4
Q

What investigations would you do and what results would you expect for a haemolytic anaemia?

A
  • FBC shows normocytic anaemia
  • Blood film schistocytes
  • Direct Coombs test is positive in autoimmune haemolytic anaemia
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5
Q

What is the most common inherited haemolytic anaemia in northern Europeans?

A

Hereditary spherocytosis

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6
Q

What is hereditary spherocytosis?

A

An autosomal dominant condition

causing sphere-shaped RBCs

that are fragile and easily break down when passing through the spleen

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7
Q

How does hereditary spherocytosis present?

A
  • jaundice
  • gallstones
  • splenomegaly
  • most notabily aplastic crisis in the presence of parvovirus
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8
Q

How is hereditary spherocytosis diagnosed?

A
  • family history
  • clinical features
  • spherocytes on the blood film
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9
Q

What investigation results would you expect to see in hereditary spherocytosis?

A
  • MHCH (Mean Corpuscular Haemoglobin Concentration) is raised on full blood count
  • Reticulocytes will be raised due to rapid turnover of RBCs
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10
Q

How is hereditary spherocytosis managed?

A

folate supplementation

splenectomy

cholecystectomy may be req. if gallstones are a problem

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11
Q

What is hereditary elliptocytosis?

How does it present?

How is it managed?

A

An autosomal dominant condition that causes RBC to be ellipse shaped.

Presntation and management are the same as hereditary spherocytosis.

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12
Q

What groups of patients is G6PD deficiency most commonly seen in?

What are the common triggers?

A

Mediterranean and African patients

It causes crises triggered by:

  1. infections
  2. medications
  3. fava beans
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13
Q

What are the types of autoimmune haemolytic anaemia?

Which is more common?

A

Warm type and cold type

Warm type more common

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14
Q

Causes of warm and cold type autoimmune haemolytic anaemia?

A

Warm is usually idiopathic

Cold is usually secondary to other conditions such as lyphoma, leukaemia, SLE and infections such as mycoplasma, EBV, CMV and HIV

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15
Q

What is alloimmune haemolytic anaemia?

A

Occurs either when there is foreign RBCs circulating destroyed by an immune reaction or foreign antibodies that destroy native RBCs causing haemolysis.

Either

  1. Transfusion reactions
  2. Haemolytic disease of the newborn
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16
Q

What is paroxysmal nocturnal haemoglobinuria?

How does it present?

A

the complement cascade acts on the RBCs causing haemolysis

Presentation:

  • red urine in the morning containing
    • haemoglobin
    • haemosiderin
  • anaemic
  • predisposed to thrombus (DVT, PE, hepatic vein thrombus)
  • predisposed to smooth muscle dystonia (oesophageal spasm and erectile dysfunction)
17
Q

Useful way to imagine microangiopathic haemolytic anaemia?

Usually secondary to which conditions?

A

Imagine a mesh inside the small blood vessels shredding the RBCs.

Secondary to

  1. Haemolytic Uraemic Syndrome (HUS)
  2. Disseminated Intravascular Coagulation (DIC)
  3. Thrombotic thrombocytopenia purpura (TTP)
  4. SLE
  5. Cancer
18
Q

What is the management of prosthetic valve haemolysis?

A
  • monitoring
  • oral iron
  • blood transfusions if severe
  • revision surgery may be required in severe cases

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