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Paediatrics > Haematology > Flashcards

Haematology Flashcards

1
Q

Briefly describe the pathophysiology behind Von Willebrand disease.

A

Bleeding disorder due to an abnormality of vWF. vWF acts as an adhesive bridge between platelets and the damaged sub-endothelium. Without it it takes longer for bleeding to stop.

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2
Q

Describe the treatment for VWD.

A
  1. Mild- Desmopressin (DDAVP)

2. Severe- plasma dereived FVIII concentrate

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3
Q

Describe the inheritance pattern seen in haemophilia.

A

X linked recessive

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4
Q

Haemophilia A is due to a deficiency in which clotting factor?

A

Factor VIII

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5
Q

Haemophilia B is due to a deficiency in which clotting factor?

A

Factor IX

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6
Q

Give 3 signs of haemophilia.

A
  1. Easy bruising
  2. Haematomas
  3. Joint bleeds - prophylaxis should be given to prevent this.
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7
Q

Give 2 causes of thrombocytopenia in children.

A
  1. ITP

2. Marrow failure

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8
Q

Give 3 signs of thrombocytopenia.

A
  1. Petechial rash.
  2. Bruising.
  3. Bleeding.
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9
Q

What diagnosis would you suspect in a child with a single figure platelet count but is otherwise well?

A

ITP.

They would have a normal blood film and clotting, just very low platelets.

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10
Q

What can trigger acute ITP?

A

Viral infection

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11
Q

What is the most common cause of anaemia in children?

A

Iron deficiency

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12
Q

Give 3 signs of anaemia in children.

A
  1. Pallor.
  2. Irritable.
  3. Tachycardic.
  4. Murmurs.
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13
Q

What is the treatment for iron deficiency anaemia?

A

Ferrous sulphate tablets

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14
Q

Why does the treatment for iron deficiency anaemia sometimes fail?

A

Due to non-compliance

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15
Q

Give 3 signs of beta thalassaemia major.

A
  1. Severe anaemia.
  2. Jaundice.
  3. Splenomegaly.
  4. Failure to thrive.
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16
Q

Describe the management of beta thalassaemia major.

A
  • Genetic counselling.
  • Blood transfusions.
  • Iron chelation.
17
Q

What does a low reticulocyte count indicate?

A

A production problem e.g. infection, renal disease, drugs, marrow failure/infiltration

18
Q

What does a high reticulocyte count indicate?

A

A degradation problem e.g. bleeding or haemolysis

19
Q

Give 3 consequences of sickle cell disease.

A
  1. Anaemia.
  2. Infection.
  3. Painful crises.
  4. Stroke.
  5. Acute chest syndrome
  6. Splenic sequestration.
20
Q

Describe the inheritance pattern seen in sickle cell disease.

A

Autosomal Recessive.

21
Q

What is the effect of sickle cell disease on Hb and reticulocyte count?

A

Low Hb.

Raised reticulocyte count.

22
Q

Describe the treatment for sickle cell disease.

A
  • Hydroxycarbamide.
  • Transfusions.
  • Stem cell transplants
23
Q

What can trigger spherocytosis?

A

Parvovirus infection

24
Q

Describe the inheritance pattern seen in hereditary spherocytosis

A

Autosomal Dominant

25
Q

How can spherocytosis be treated?

A

Splenectomy - can increase RBC survival

26
Q

What type of leukaemia is most common in children?

A

ALL

27
Q

Give 3 symptoms of ALL.

A

Bone marrow failure:

  • anaemia: lethargy and pallor
  • neutropaenia: frequent or severe infections
  • thrombocytopenia: easy bruising, petechiae

And other features:

  • bone pain (secondary to bone marrow infiltration)
  • splenomegaly
  • hepatomegaly—fever is present in up to 50% of new cases (representing infection or constitutional symptom)
  • testicular swelling
28
Q

Describe the treatment for ALL.

A

Chemotherapy.
CNS directed therapy.
Stem cell transplant.

29
Q

Which features suggest poor prognostic features of ALL?

A 
Age < 2 years or > 10 years
WBC > 20 * 109/l at diagnosis
T or B cell surface markers
Non-Caucasian
Male sex
30
Q

What investigations might you do in a child to determine whether they have ALL?

A
  1. Blood film.
  2. Serum chemistry.
  3. CXR.
  4. BM aspirate.
  5. Lumbar puncture - see if disease is in the CSF.
31
Q

What is HSP?

A

A systemic vasculitis where there is deposition of IgA complexes. It is characterised by a palpable purpura and there is often renal involvement.

32
Q

What is the SE of transfusions in beta thalassemic major patients? How is it managed?

A

repeated transfusion → iron overload

Tx: s/c infusion of desferrioxamine / oral iron chelation

Paediatrics (21 decks)

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