CP42 - paediatric haematology

Question 1

what are the difference between child and adult haematology?

Answer 1

Site of haematopoeisis varies

Haemoglobin switching (from Hb-F to Hb-A)

At birth 55-65% is HbF

Difference in red cell structure and metabolism

Larger red blood cells (greater O2 affinity)

Higher haematocrit (measure of the viscosity or ‘thickness’ of the blood. )

Rapidly reach similar values to adults

Question 2

what are the difference in WBC in adult and child?

Answer 2

Similar numbers - higher lymphocyte counts

Immune system is a work in progress
IgG crosses the placenta

breast milk cover every other antibodies

Question 3

what are the difference in platelet in adult and child?

Answer 3

reach adult by 18/40 gestation

initially larger, by birth slim down to adult size

functionally different at birth - hyporesponsive to certain agonists, hyperresponsive to vWF

eventually balance out

baby very good at clotting

Question 4

what are the difference in haemostasis in adult and child?

Answer 4

present but imperfect at birth

coagulation proteins do not cross placenta effectively

only fibrinogen, FV, FVIII, FXIII are normal at birth

most haemostatic parameters reach adult by 6 months

Question 5

what are some examples for congenital anaemia in childhood

Answer 5

haemoglobin synthesis prob - haemoglobinopathy

bone marrow failure syndrome

Bone marrow infiltration

peripheral destruction of the RBC

blood loss

Question 6

what are some examples of haemoglobinopathy?

Answer 6

sickle cells and thalamaemia

Question 7

how can haemoglobinopathy be prevent?

Answer 7

antenatal (before birth) screening

Question 8

what can cause peripheral destruction leading to congenital anaemia

Answer 8

Rh / ABO or other incompatibility

Membrane defect: hereditary spherocytosis

Enzyme defect: G6PD deficiency, PK deficiency

Infection

Question 9

what are some examples for acquired anaemia

Answer 9

Nutritional deficiency: iron, B12, folate

Bone marrow failure

Bone marrow infiltration

Peripheral destruction: haemolysis

Blood loss

Question 10

what are the congenital factors which can lead to bleeding and bruising?

Answer 10

platelet problem, clotting factor problem, connective tissue problem

Question 11

what are some acquired factors which can lead to bleeding and bruising?

Answer 11

Trauma: accidental, non-accidental

Tumour

Infection: acute eg meningococcus, chronic eg HIV

Immune disorder: primary - immune thrombocytopenia, TTP
secondary - SLE, ALPS

Bone marrow failure

Drug related