Paediatric Haematology

Question 1

Site of haematopoeisis in children

Answer 1

liver

Question 2

Site of haematopoeisis in adults

Answer 2

bone marrow

Question 3

What does haematocrit take into account?

Answer 3

Size of RBCs and concentration of Hb

Question 4

Describe WBCs in children

Answer 4

More lymphocytes in children
IgG crosses the placenta, IgM does not
Breast milk has all antibodies
Start producing own antibodies at 2-3 months
Vaccinations start at 2 months but require regular boosters

Question 5

When do foetuses reach adult numbers of platelets?

Answer 5

18 weeks- important to develop early as biggest risk to foetus during birth is bleeding

Question 6

Describe the responsiveness of children’s platelets to VWF

Answer 6

Hyperresponsive

Question 7

Which clotting factors are normal at birth?

Answer 7

Fibrinogen, FV, FVIII, FXIII

Question 8

When do clotting factors reach adult values?

Answer 8

By 6 months

Question 9

What is haemorrhagic disease of the newborn and why does it come about?

Answer 9

foetal vit. K is 10% of mothers- increase risk of bleeding

Question 10

Describe the concentration of coagulation inhibitors in children

Answer 10

Decreased 9ATIII, heparin, protein C, protein S

Question 11

Describe the relative concentrations of D dimers and vWF in children

Answer 11

Increased

Question 12

Describe the 3 most common causes of peripheral destruction of RBCs causing anaemia in childhood

Answer 12

Haemaglobinopathy
Peripheral destruction
Blood loss

Question 13

Describe 2 autoimmune haemaglobinopathy in neonates and children

Answer 13

Rh incompatibility- can cross placenta

ABO incompatibility- can’t corss placenta so rarely a problem

Question 14

Describe a membrane defect which can cause anaemia in children

Answer 14

Hereditary sperocytosis

Question 15

Describe 2 enzyme defects associated with anaemia in children

Answer 15

G6PD deficiency

Pyruvate kinase deficiency

Question 16

2 examples of how blood loss in children/foetuses can come about

Answer 16

Twin to twin transfusion

Fetomaternal haemorrhage

Question 17

What is the most common nutritional deficiency in children; iron, B12 or folate

Answer 17

Iron

Question 18

Give 5 causes of acquired childhood anaemia

Answer 18

Nutritional deficiency
Bone marrow failure
Bone marrow infiltration
Peripheral destruction; haemolysis
Blood loss

Question 19

Give 3 causes of congential bleeding problems in children

Answer 19

Platelet problems
Clotting factor problems
Connective tissue disorders (Ehler’s danlos)

Question 20

Give 6 causes of acquired bleeding problems in children

Answer 20

Trauma
Tumour
Infection
Immune disorder
Bone marrow fialure
Drug related

Question 21

Give 2 examples of infections in children that can cause acquired bleeding problems

Answer 21

Meningococcus

HIV

Question 22

Give 2 examples of primary immune disorders in children that can cause bleeding and bruising

Answer 22

Immune thrombocytopenia

Thrombotic thrombocytopenic purpura

Question 23

Give 2 examples of secondary immune disorders in children that can cause bleeding and bruising

Answer 23

SLE

ALPS-autoimmune lyphoproliferative syndrome

Question 24

Mode of inheritence for autoimmune lymphoproliferative syndrome

Answer 24

AD