Paediatric Haematology

Question 1

What is the pathogenesis of Anaemia?

Answer 1

1) Blood loss: Acute haemorrhage/chronic gut bleeding
2) Decreased Production: Nutritional deficiency, bone marrow failure, infiltration
3) Increased consumption: Acquired/Inherited

Question 2

What are the main blood parameters?

Answer 2

1) HB (Normal for age range and sex)
2) Abnormality in single cell/multiple cell lines
3) MCV (Microcytic, normocytic, macrocytic)

Question 3

What are some examples of MCV causes?

Answer 3

Micro: Iron def, Thalassaemia, sideroblastic
Normal: Acute blood loss, infection, renal failure, bone marrow infiltration
Macro: Aplastic anaemia, newborn

Question 4

What do different reticulocyte levels indicate?

Answer 4

Low: Production problem e.g. Infection, renal disease, drugs, marrow failure/infiltration
High: Degradation problem e.g. bleeding/haemolysis

Question 5

What is the most common cause of childhood anaemia?

Answer 5

Iron deficiency

Question 6

What are the main signs of anaemia?

Answer 6

1) Pallor
2) Irritable
3) SOB
4) Lethargy
5) Tachycardic

Question 7

What is treatment for IDA?

Answer 7

Ferrous Sulphate Tablets/Oral Iron
- Fails due to non-compliance

Question 8

What causes increased destruction of RBC?

Answer 8

IntraCorpuscular: Hb, Enzyme or Membrane
ExtraCorpuscular: Autoimmune, fragmentation, hypersplenism and plasma factors

Question 9

What are the main presentations of G6PD?

Answer 9

1) Neonatal jaundice
2) Chronic non-spherocytic haemolytic anaemia
3) Intermittent episodes of intravascular haemolysis
(X Linked recessive)

Question 10

What are two types of Haemolysis?

Answer 10

1) Intravascular Haemolysis: Haemoglobinuria, rigors, fever, back pain
2) Sporadic Haemolysis: Drugs, fava beans, fever, acidosis
TREAT: Stop precipitant, transfusion and renal support

Question 11

What is the inheritance pattern of SCD?

Answer 11

Autosomal recessive

Question 12

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

Answer 12

Low Hb.
Raised reticulocyte count.

Question 13

What is the treatment for SCD?

Answer 13

1) Hydroxycarbamide
2) Transfusions
3) Stem cell transplants

Question 14

What are the main problems of SCD?

Answer 14

Anaemia.
Infection.
Painful crises.
Stroke.
Acute chest infection/infarction.
Splenic sequestration

Question 15

Spherocytosis: Cause, pattern and treatment?

Answer 15

C: Parovirus
P: AD
T: Splenectomy (^ RBC survival)

Question 16

Name 2 coagulopathies

Answer 16

1) Von Willebrand Disease
2) Haemophilia

Question 17

Describe VWD PP

Answer 17

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.

Question 18

How is VWD inherited, presented and treated

Answer 18

In: AD/AR
P: Easy bruising, epistasis
Diagnosis: Clotting screen showing prolonged APTT (Affects factor 8!)#
Treat: No cure, instead manage with: Tranexamic acid, desmopressin, nebulised

Question 19

Haemophilia: Pattern, Clotting factor, Presentation

Answer 19

Pattern: X Linked Recessive
Type A: Factor 8 (VIII) deficiency
Type B: Factor 9 deficiency
Presentations: Easy bruising, Haematoma, mouth/joint bleeds

Question 20

What are 2 causes of Thrombocytopenia?

Answer 20

1) Marrow failure
2) ITP

Question 21

What are 3 signs of Thrombocytopenia?

Answer 21

1) Petechial rash
2) Bruising
3) Bleeding

Question 22

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

Answer 22

ITP –> Low platelets, normal blood film and clot

Question 23

What can trigger acute ITP?

Answer 23

Viral infection

Question 24

Give 3 signs of beta thalassaemia major.

Answer 24

Severe anaemia.
Jaundice.
Splenomegaly.
Failure to thrive.

Question 25

Describe the management of beta thalassaemia major.

Answer 25

Genetic counselling.
Blood transfusions.
Iron chelation.