Paediatric Haematology

Question 1

Why does physiological anaemia of infancy occur?

Answer 1

High haemoglobin levels at birth mean high oxygen delivery to the tissues at birth. This has a negative feedback effect on EPO so there is reduced production of haemoglobin by the bone marrow

Question 2

What are some reasons that cause premature neonates to becomes anaemic?

Answer 2

Less time in utero receiving iron from the mother
RBC production cant keep up with rapid growth in first few weeks
Reduced EPO levels
Blood tests remove from their circulating volume

Question 3

What are the two most common causes of anaemia in older children?

Answer 3

Iron deficiency and blood loss

Question 4

What are causes of microcytic anaemia? (TAILS)

Answer 4

Thalassaemia, anaemia of chronic disease, iron deficiency, lead poisoning, sideroblastic anaemia

Question 5

What are causes of normocytic anaemia? (3As and 2Hs)

Answer 5

Acute blood loss, aplastic anaemia, anaemia of chronic disease
haemolysis, hypothyroidism

Question 6

What are causes of macrocyctic anaemia?

Answer 6

B12 and folate deficiencies, hypothyroidism, alcohol, reticulocytosis,

Question 7

What are symptoms of anaemia?

Answer 7

Tiredness, palpitations, breathlessness, headaches, dizziness

Question 8

What are some specific signs that would indicate iron deficiency anaemia?

Answer 8

Pica, hair loss/brittle hair and nails, koilonychia, angular chelitis, atrophic glossitis

Question 9

Jaundice occurs with which cause of anaemia?

Answer 9

Haemolytic anaemia

Question 10

Bone deformities are more associated with which cause of microcytic anaemia?

Answer 10

Thalassaemia

Question 11

From most to least common list the types of leukaemia seen in children

Answer 11

Acute lymphoid leukaemia
Acute myeloid leukaemia
Chronic myeloid leukaemia

Question 12

What ages are ALL and AML most commonly seen?

Answer 12

ALL common between 2-5 but peaks between 2-3
AML peaks under 2 years of age

Question 13

What is the pathophysiology of leukaemia and why does this lead to pancytopenia?

Answer 13

A genetic mutation in a precursor cell in the bone marrow lead to excessive production of a single type of abnormal white blood cell.
This leads to suppression of the other cell lines leading to pancytopenia

Question 14

What is pancytopenia a combination of?

Answer 14

Anaemia
Leukopenia
Thrombocytopenia

Question 15

What is the main environmental risk factor for leukaemia?

Answer 15

Radiation in utero e.g from abdominal X-ray of mum while pregnant

Question 16

What are some other conditions which can predispose someone to a higher risk of developing leukaemia?

Answer 16

Downs, kleinfelter syndrome, fanconi’s anaemia, noonan syndrome

Question 17

Although the presentation of leukaemia can be quite non-specific, what are some symptoms?

Answer 17

Leukopenia: recurrent infections
Thrombocytopenia: bleeding and abnormal bruising
Anaemia: fatigue, pallor
Unexplained fever, failure to thrive, weight loss, night sweats, bone pain, hepatosplenomegaly, generalised lymphadenopathy

Question 18

If leukaemia is suspected then what investigation would be requested and in what timeframe?

Answer 18

Full blood count within 48 hours

Question 19

What investigations can be done to establish the diagnosis of leukaemia?

Answer 19

Full blood count (anaemia, leukopenia, thrombocytopenia)
Blood film (blast cells)
Bone marrow biopsy
Lymph node biopsy

Question 20

What further tests can be done to stage the leukaemia?

Answer 20

Chest X ray
CT scan
Lumbar puncture
Genetic analysis of abnormal cells

Question 21

What is sickle cells disease?

Answer 21

A genetic condition that causes sickle shaped red blood cells which are more likely to stick together and be easily destroyed

Question 22

What is the inheritance pattern of sickle cell disease?

Answer 22

Autosomal recessive

Question 23

What is the relationship with sickle cell disease and malaria?

Answer 23

Sickle cell is more common in people from areas traditionally affected by malaria and having one copy of the gene (sickle cell trait) reduces the severity of malaria

Question 24

When are babies first screened for sickles cell disease?

Answer 24

Newborn blood spot test on day 5

Question 25

What are some complications of sickles cell disease?

Answer 25

Anaemia, acute chest syndrome, sickle cell crisis, chronic kidney disease, increased risk of infection, stroke, avascular necrosis

Question 26

What is priapism?

Answer 26

Persistent and painful erection occurring when blood gets trapped in the penis- urological emergency treated by aspirating blood from penis

Question 27

Although sickle cell crises can occur spontaneously, what are some triggers?

Answer 27

Dehydration, infection, cold weather or stress

Question 28

How are sickle cell crises managed?

Answer 28

Analgesia, fluids, keep warm
Admit to hospital
Treat any underlying infections

Question 29

Infection with which virus usually triggers an aplastic crisis?

Answer 29

Parvovirus B19

Question 30

How will acute chest syndrome present and what will be seen on chest x ray?

Answer 30

Fever, dyspnoea, chest pain, cough and hypoxia
Pulmonary infiltrates

Question 31

what are the general principles of management for sickle cell disease?

Answer 31

Avoid triggers
Stay up to date with vaccinations
Prophylactic ABx
Hydroxycarbamide
Crizanlizumab
Blood transfusions for severe anaemia
Bone marrow transplant (can be curative)

Question 32

Why do we give hydroxycarbamide in sickle cell disease?

Answer 32

Promotes production of fetal Hb which does not lead to sickling of RBCs.
It reduces vaso-occlusive crises and anaemia

Question 33

Why do we use crizanlizumab for sickle cell disease?

Answer 33

This monoclonal antibody targets p-selectin which is an adhesion molecule on endothelial cells and platelets.
Prevents RBCs sticking to blood vessel walls and reduces vaso-occlusive crises

Question 34

What is the likely underlying diagnosis for a patient who develops anaemia and becomes jaundice after eating broad beans/developing an infection or being treated with anti-malarials?

Answer 34

G6PD deficiency

Question 35

Is lymphoma more commonly seen in younger or older children?

Answer 35

Older children