Paediatric Haematology

Question 1

What sites does haemopoiesis occur throughout the paediatric lifespan?

Answer 1

0-2 1/2 months - yolk sac
7 weeks to 7months - liver and spleen
Birth onwards - bone marrow

LIVER AND SPLEEN CAN BE RECRUITED WITH PATHOLOGY

Question 2

What is the name by which the liver and spleen are re-recruited during pathological circumstances?

Answer 2

Extra-medullary haemopoiesis

Question 3

What are the structural/function differences in HbF vs HbA?

Answer 3

• large spherical red balls
• Higher O2 affinity
• Higher hematocrits (which normalises to adult levels around 3 moths)

Question 4

At birth what is the % of Hb F in the body?

Answer 4

55-65%

Question 5

What chromosome codes for the alpha chains?

Answer 5

Chromosome 16

Question 6

What chromosome codes for the beta chains?

Answer 6

Chromosome 11

Question 7

What are the different Hb types during Hb switching?

Answer 7

1st trimester
zita2 epsilon2 = Hb gower

Zita2 gamma2 = Hb Portland

Alpha2 epsilon2 = Hb Gower 2

2nd trimester

alpha2 gamma2 = HbF

Neonatal
Alpha2 beta2 = HbA

Aphla 2 delta 2 = HbA2

Question 8

Are lymphocytes higher or lower in child vs adult? Why?

Answer 8

Higher - immune centre = work in progress. Exposure to lots of different things e.g. pathogens, food

Question 9

What Ig crosses the placenta?

Answer 9

IgG

Question 10

What Ig does great milk contain>

Answer 10

IgA, IgD, IgEm IgG, IgM

Question 11

What type of immunity is great milk providing?

Answer 11

Passive immunity

Question 12

When do babies start making antibodies?

Answer 12

2-3.5 months

Question 13

When do babies start making satisfactory immune response

Answer 13

6-12 months

Question 14

When do we start giving vaccinations to babies?

Answer 14

2 months boosts immune system

Question 15

Fetal platelets by the 1st trimester are around the same quantity as adults. Why?

Answer 15

Bleeding = biggest risk to foetus. Body prioritises on platelet production

Question 16

What are the functional differences between adult platelets vs fatal?

Answer 16

Fetal platelets = more response to vWf but hyporespontsive to some agonists

Question 17

What coagulation factors are in normal quantities at birth?

Answer 17

Fibrinogen
V
VIII
XIII

(others normalise in 6 months)

Question 18

What coagulation factors are bit K dependent?

Answer 18

II, VII, IX, X

Protein C and S

Question 19

What are babies given to prevent haemorrhagic disease of the newborn?

Answer 19

Vit K injections

Question 20

What coagulation factors are deficienct in the new born?

Answer 20

Factors VII, IX, X, XI, XII

Prothrombin (II)

Question 21

How is neonatal haemostasisi different to adult?

Answer 21

Reduced conc of coagulation inhibitors (reduced ATIII, Heparin cofactor, protein C and S)

• Raised vWf
• reduced coagulation factors
  (II, VII, IX, X, XI, XII)
• different platelet aggregation

Question 22

What is hereditary spherocytosis?

Answer 22

Brittle red blood cells - lyse (jaundice/billirubin)