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YEAR 4-PAEDIATRICS > Haematology > Flashcards

Haematology Flashcards

1
Q

What is the function of haemoglobin?

A

Haemoglobin is the molecule responsible for transporting oxygen around the body.

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2
Q

Where is haemoglobin found?

A

In red blood cells.

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3
Q

In adults, where does haemoglobin ‘pick up’ oxygen?

A

**Haemoglobin picks up oxygen in the lungs.
**
*
Then transports it through the blood and releases it into the tissues. *

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4
Q

In the fetus, where does haemoglobin pick up oxygen?

A

Haemoglobin picks up oxygen in the placenta, ‘stealing’ it from the mothers haemoglobin.

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5
Q

How many subunits does haemoglobin have?

A

Haemoglobin has **4 protein subunits. **

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6
Q

What 4 subunits does adult haemoglobin (HbA) have?

A

2x Alpha subunits
2x Beta subunits

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7
Q

What 4 subunits does fetal haemoglobin (HbF) have?

A

2X Alpha subunits
2x Gamma subunits

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8
Q

What does the difference in subunits between fetal and adult haemoglobin allow for?

A
  • The structure gives fetal haemoglobin a greater affinity to oxygen then adult haemoglobin.
  • This means the oxygen binds to fetal haemoglobin more easily and is more relectuant to let go (than adult haemoglobin).
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9
Q

Explain why it is important that fetal haemoglobin has a greater affinity to oxygen than adult haemoglobin?

A

It allows fetal haemologin to ‘steal’ oxygen away from the adult maternal haemoglobin in the placenta.

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10
Q

What would happen if fetal and adult haemoglobin had the same affinity for oxygen?

A

If fetal and adult haemoglobin had the same affinity for oxygen then the fetal haemoglobin would not be able to take any oxygen from the adult haemoglobin.

The fetal blood would therefore hvae no oxygen, to provide to tissues.

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11
Q

What is a oxygen dissociation curve?

A

A graph which shows the relationship between the partial pressure of oxygen (how much oxygen is in a space) and the saturation of haemoglobin with oxygen (how ‘full’ the haemoglobin is).

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12
Q

What does a higher partial pressure suggest?

A

The higher the partial pressure the more oxygen there is in the area.

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13
Q

What does a higher saturation of haemoglobin with o2 suggest.

A

The higher the saturation of haemoglobin with O2, the more oxygen there is bound to the haemoglobin.

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14
Q
A
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15
Q

How many oxygen molecules can bind to haemoglobin?

A
  1. Each oxygen molecules binds to the haem group on each of the 4 subunits.
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16
Q

Explain the relationship between partial pressure of oxygen and the saturation of haemoglobin with oxygen as seen in the graph.

A

As the partial pressure of oxygen increases, the saturation of haemoglobin with oxygen increases.

AKA;
As the partial pressure of oxygen goes up, more oxygen will be bound to haemoglobin until it is completely saturated.

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17
Q

Explain the difference between adult haemoglobin and fetal haemglobin with reference to partial pressure of oxygen as seen on the graph.

A

Adult haemoglobin requires a higher partial pressure of oxygen to completely saturate the haemoglobin with oxygen compared with fetal haemoglobin.

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18
Q

Explain why adult haemoglobin requires a higher partial pressure of oxygen to completely saturate the haemoglobin with oxygen compared to fetal haemoglobin.

A

This is because fetal haemoglobin has 2x gamma subunits which gives it a greater affinity for oxygen, so it can more easily bind oxygen, and therefore at lower concentrations.

Whereas adult haemoglobin has 2x beta units which gives the molecules a lesser affinity for oxygen compared with fetal haemoglobin, so greater concentrations of oxygen are required to saturate the haemoglobin.

I think- not 100% on this.
Essentially, adult haemoglobin has a lower affintiy for oxygen so requires a higher partial pressure of oxygen to completely saturate the molecule.

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19
Q

At what weeks gestation does production of fetal haemoglobin start to decrease?

A

32-36 weeks

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20
Q

At what weeks gestation does the fetus start to produce adult haemoglobin?

A

32-36 weeks.
As the production of fetal haemoglobin decreases, adult haemoglobin is produced in greater quantities.

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21
Q

True or false.
At birth, around half the haemoglobin produced is HbF and half is HbA?

A

True

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22
Q

What does HbF stand for?

A

Fetal haemoglobin

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23
Q

What does HbA stand for?

A

Adult haemoglobin

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24
Q

At what age do the red blood cells contain entirely adult haemoglobin (HbA)?

A

6 months+
At this age very little fetal haemoglobin is produced.

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25
Why does fetal haemoglobin **not** lead to 'sickling' of red blood cells?
Because in sickle cell diease there is a genetic abnormality coding for the **beta** subunit in the haemoglobin causing the sickle shape of the red blood cells. But in fetal haemoglobin there are **no beta subunits**, just alpha and gamma, so there is no 'sickling' of the red blood cells.
26
Fetal haemoglobin **cannot** lead to the sickling of red blood cells. Therefore increasing the production of fetal haemoglobin can be used in patients with sickle cell dsiease. What treatment is given to increase the production of fetal haemoglobin?
Hydroxycarbamide (Increases the production of fetal haemoglobin)
27
What is Sickle Cell anaemia?
A genetic condition that causes sickle (cresent) shaped red blood cells.
28
Patients with sickle-cell disease have an abnormal varient of haemoglobin called what?
Haemoglobin S (HbS)
29
Is Sickle Cell anaemia autosomal dominant or recessive?
It is an autosomal recessive condition.
30
What gene does sickle cell anaemia affect?
The gene for beta-globin on chromosome 11.
31
If a individual only has 1 abnormal copy of the gene, what is this condition called?
Sickle-cell trait. They are carriers of the condition and are usually asymptomatic.
32
If an individual has 2 copies of the abnormal gene, what is the condition called?
Sickle- cell disease
33
What is the relationship between sickle cell disease and malaria?
-Having one copy of the abnormal gene (sickle cell trait) reduces the severity of malaria. -Therefore individuals with sickle cell trait are more likely to survivce malaria and thus pass on their genes. -Therefore sickle cell disease is more common in areas affected by malaria, as it is a selective advantage.
34
Sickle cell disease is screened for. When is it screen for?
It is tested for on the **newborn blood spot** screening test at around **5 days old. **
35
If a pregnant woman is considered high risk of being a carrier of the sickle cell gene is she offered any testing?
yes, pregnant women considered high risk are offered a blood test before 10 weeks of pregnancy to test for the sickle cell gene.
36
What is a sickle cell crisis?
A sickle cell crisis is a painful episode that occurs when red blood cells in people with sickle cell disease change shape and block blood vessels. This can cause severe pain that lasts for days or weeks.
37
Sickle cell crisis can occur spontaneously or can be triggered. Suggest 3 triggers for a sickle cell crisis.
1. Dehydration 2.Infection 3. Stress
38
Suggest 4 symptoms of a sickle cell crisis.
1. Severe pain (usually affects the back or limbs) 2. Shortness of breath 3. Fatigue 4.Dizziness
39
What is the treatment for Sickle cell crisis?
There is no specific treatment for sickle cell crisis. Treatment is supportive. E.g. IV fluids for dehydration, analgesia, keep warm.
40
What is the most common type of sickle cell crisis?
**Vaso-occlusive Crisis** *AKA, Painful Crisis*
41
Vaso-occlusive crisis is a type of sickle cell crisis. It is caused by the sickle-shaped red blood cells clogging capillaries and causing distal ischaemia. It can cause priapism, what is this and what is the treatment?
Priapism is a prolonged erection of the penis, it is a urological emergency and is treated by aspirating blood from the penis. Nb- Sickle cell crisis, causes the blood to become trapped in the penis causing a painful and persistent erection.
42
What is a splenic sequestation crisis?
* A type of sickle cell crisis, whereby red blood cells block the blood flow within the spleen. * This causes an acutely enlarged and painful spleen. * The blood pools in the spleen as it is blocked causing severe anaemia and hypovolaemic shock.
43
Splenic sequestration crisis is considered an emergency. What is the treatment?
-Blood transfusions -Fluid resuscitation -Splenectomy may be considered in recurrent cases as it is curatvie of splenic sequestration crises.
44
What is a complication of splenic sequestration crisis?
Susceptibility to infections. Splenic sequestation crisis can lead to splenic infarction, causing hypospelnism (impaired spleen function) and thus increase the susceptibility to infection.
45
What is a aplastic crisis?
A (sickle cell) crisis when the bone marrow temporarily stops producing new red blood cells.
46
Aplastic crisis is usually triggered by an infection with what?
Parovirus B19
47
What is the treatment for aplastic crisis?
Supportive, blood transfusions if necessary. Usually resolves spontaneously within a week.
48
Acute chets syndrome is a complication of sickle-cell disease. What is it?
Acute chest syndrome occurs when the vessels supplying the lungs become blocked with red blood cells. Patients will present with fever, SOB, chest pain, cough.
49
What will the chest x-ray of somone with acute chest syndrome show?
Pulmonary infiltrates
50
Acute chest syndrome is a medical emergency with high mortality rates. Treatment is supportive, suggest 4 treatments.
1. Analgesia 2. IV fluids 3. Blood Transfusions 4. Respiratory support with oxygen.
51
A specialist MDT will manage sickle cell disease. Suggest 6 features of management.
1. Avoid triggers for crisis 2. Up to date vaccinations 3. Treatment with Hydroxycarbamide (to stimualte fetal haemoglobin) 4. Blood transfusions (for severe anaemia) 5. Monoclonal antibodies e.g. Crizanlizumab 6. Bone marrow transplant (can be curative)
52
Explain how Crizanlizumab helps in sickle cell disease?
* **Crizanlizumab** is a monoclonal antibody that taregts P-selectin. * P-selectin is an adhesion molecule found on endothelial cells on the inside of blood vessels and platelets. * By targeting P-selectin is prevents red blood cells from sticking to the blood vessel wall.
53
What is Leukaemia?
Leukaemia is a type of blood cancer that affects blood cells in the bone marrow. Typically white blood cells. There are various types of leukaemia.
54
Types of leukaemia can be classified depending on how rapidly they progress (acute or chronic) and the cell line that is affected (myeloid or lymphod) Suggest 3 types of leukaemia that affect children.
1. Acute lymphoblastic leukaemia (ALL) 2. Acute myeloid leukaemia (AML) 3. Chronic myeloid leukaemia (CML)
55
At what age does Acute lymphoblastic leukaemia (ALL) peak?
Aged 2-3 years
56
At what age does acute myeloid leukaemia (AML) peak?
Under 2 years
57
Leukaemia is a form of cancer of the cells in the bone marrow. A genetic mutation in one of the precursor cells in the bone marrow leads to excessive production of a single type of abnormal white blood cell. The excessive production of a single type of cell can lead to suppression of the other cell lines, causign underproduction of other cell types. This results in **pancytopenia. ** What is pancytopenia?
A combination of low.... -Red blood cells -White blood cells -Platelets
58
Suggest 3 risk factors for leukaemia?
1. Radiation exposure (e.g during pregnancy) 2. Down's Syndrome 3. Kleinfelter Syndrome
59
The presentation of leukaemia is typically non-specific. Suggest 6 features of leukaemia
1. Abnormal bruising/petechiae (thrombocytopenia) 2. Night sweats 3. Weight loss 4. Unexplained fever 5. Hepatosplenomegaly 6. Failure to thrive
60
Suggest 4 investigations to help diagnose Leukaemia?
1. Full blood count (would show anaemia, leukopenia, thrombocytopenia and high number of the abnormal WBCs) 2. Blood film 3. Bone marrow biopsy 4. Lymph node biopsy *Further testing e.g. chest x-ray, CT, maybe needed for staging *
61
What is a) Anaemia b) Leukopenia c) Thrombocytopenia
a)Anemia is low red blood cells b)Leukopenia is low white blood cells C)Thrombocytopenia is low platelets
62
Treatment of leukaemia will be coordinated by a paediatric oncology MDT. What is the primary treatment for leukaemia?
Chemotherapy
63
Suggest 4 complications of chemotherapy?
1. Stunted growth and development 2. Immunideficieny and infection 3. Neurotoxicity 4. Infertility
64
What is Tumour lysis syndrome?
A life threatening complication of certain haematological malignancies most often seen at the start of chemotherapy treatment, when there is rapid breakdown of high numbers of cancer cells.
65
Tumour lysis syndrome can cause release of intracellular contents into the blood, what 3 things are released intot he blood.
Raised potassium, phosphate and nucelic acids.
66
What can the release of intracellular contents in tumour lysis syndrome result in?
Impaired renal function / acute kidney injury and symptoms of electrolyte derangement (e.g. cardiac arrhythmias due to hyperkalaemia).
67
What is Thalassaemia?

YEAR 4-PAEDIATRICS (17 decks)

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