Haematology 5 - Haemolytic anaemias Flashcards

1
Q

Recall some causes of intravascular vs extravascular haemolytic anaemias

A
Intravascular: 
- Malaria (black water fever) 
- G6PDD
- MAHA (due to HUS/TTP/PNH) 
Extravascular: 
- Autoimmune
- hereditary spherocytosis
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2
Q

Which infection are patients with haemolytic anaemias more susceptibile to?

A

Parvovirus B19

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3
Q

What is the expected LDH level in haemolytic anaemia?

A

High

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4
Q

What is the inheritance pattern of hereditary spherocytosis?

A

Autosomal dominant

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5
Q

How can hereditary spherocytosis be diagnosed?

A

Examination of mean cell fluorescence

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6
Q

What is the difference in clinical features of hereditary elliptocytosis in a heterozygote vs a homozygote?

A

Heterozygote: no polychromasia
Homozygote: Severe haemolytic anaemia

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7
Q

In which parts of the world is G6PDD most common?

A

Where malaria is endemic

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8
Q

What is the inheritance pattern of G6PDD?

A

X linked

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9
Q

What is the normal physiological role of G6PD?

A

NADPH generation

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10
Q

What is the main symptom of G6PDD in neonates?

A

Jaundice

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11
Q

Recall 3 triggers for a crisis in G6PDD

A

Moth balls
Fava beans
Anti-malarials

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12
Q

What is the typical appearance of erythrocytes in Pyruvate Kinase Deficiency?

A

Kinocytes (‘spiky’)

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13
Q

What is the first investigation to do in haemolytic anaemia?

A

DAT/ Coombs test

Need to exclude autoimmune haemolysis

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14
Q

What does a positive urinary haemosiderin test show?

A

Intravascular haemolysis

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15
Q

Recall some indications for splenectomy

A
PKU
Hereditary spherocytosis
Severe elliptocytosis/ pyropoikilocytosis
Thalassaemia
Immune haemolytic anaemia
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16
Q

What is homozygous hereditary spherocytosis also known as?

A

Pyropoikilocytosis