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Haematology and Immunology > Red Cells Continued > Flashcards

Red Cells Continued Flashcards

1
Q

What does PNH stand for?

A

Paroxysmal nocturnal haemoglobinuria

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2
Q

What is PNH?

A

Acquired disorder leading to haemolysis (mainly intravascular) of haematological cells

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3
Q

Pathology of PNH

A

Increased sensitivity to the cell membranes of complement due to the lack of GPI

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4
Q

What are patients with PNH more prone to?

A

Thrombosis

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5
Q

Presentation of PNH

A 
Haemolytic anaemia
May have pancytopenia
Haemoglobinuria 
Thrombosis e.g. Budd chiari syndrome 
Some patients may develop aplastic anaemia
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6
Q

What is haemoglobinuria?

A

Classically dark coloured urine in the morning

Can occur throughout the day

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7
Q

Investigation of PNH

A

Hams test

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8
Q

What is hams test?

A

Acid induced haemolysis (normal red cells would not)

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9
Q

Treatment of PNH

A

Blood product replacement
Anticoagulation
Stem cell transplant

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10
Q

What is the most common cause of haemolytic anaemia in patients with G6PD deficiency?

A

Ciprofloxacin

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11
Q

Who with sickle cell anaemia should have a low threshold for hospital admission?

A

In a child
Person with temp over 38C (risk of rapid deterioration)
Person with chest symptoms (as acute chest syndrome may develop quickly)

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12
Q

Investigation for hereditary spherocytosis

A

EMA binding test

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13
Q

When does intravascular haemolysis occur?

A

RBCs rupture in the vessel and are bound to haptoglobin to be excreted in the urine

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14
Q

When does extravascular haemolysis occur?

A

Phagocytosis of RBCs

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15
Q

What systemic disease is a cause of autoimmune haemolytic anaemia?

A

SLE

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16
Q

How long does oral iron tablets take to work?

A

2 - 4 weeks

17
Q

How long does it take for oral iron tablets to change blood results?

A

2 - 3 months

18
Q

What is mixed anaemia and what may it present as?

A

Both vit B12 and iron deficiency anaemias

Normocytic anaemia

19
Q

What crises are recognised with sickle cell disease?

A 
Chest crisis
Thrombotic 'painful' crisis
Sequestration 
Aplastic
Haemolytic
20
Q

Thrombotic crisis is also known as what?

A

Painful crisis

Vaso-occlusive crisis

21
Q

What is thrombotic crisis precipitated by?

A

Infection
Dehydration
Deoxygenation

22
Q

Features of thrombotic crisis

A

Infarcts occur in various organs including

  • Bones (e.g. avascular necrosis of the hip)
  • hand foot syndrome in children
  • lung
  • spleen
  • brain
23
Q

Explain sequestration crisis

A

Sickling within organs such as the spleen or the lungs, causing pooling of blood with the worsening of anaemia
Increased reticulocytes

24
Q

What is the most common cause of death in sickle cell disease after childhood?

A

Acute chest crisis

25
Q

What is aplastic crisis caused by?

A

Infection with parovirus

26
Q

What does aplastic crisis cause?

A

Sudden fall in Hb

Reduced reticulocytes

27
Q

Pathology of haemolytic crisis

A

Fall in Hb due to an increased rate in haemolysis

28
Q

How often do sickle cell patients receive a pneumococcal vaccine?

A

Every 5 years

29
Q

What type of diet is a risk factor for B12 deficiency?

A

Vegan

30
Q

What anaemia does sickle cell disease cause?

A

Normocytic with increased retics

31
Q

Explain hand foot syndrome in children

A

Sickling of RBCs interfering with the circulation in the hands and feet
Results in swelling, pain and erythema with a sudden onset
Sickle cell disease

32
Q

What symptom would point towards splenomegaly?

A

“Stomach fullness”

33
Q

In all people with iron deficiency anaemia, what should they be screened for?

A

Coeliac disease

34
Q

What does a disproportionally low microcytic anaemia indicate?

A

Beta-thalassaemia trait

35
Q

What can parovirus precipitate if you have hereditary spherocytosis?

A

Haemolytic crisis

Haematology and Immunology (18 decks)

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