Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

GP Extras > Haematology > Flashcards

Haematology Flashcards

1
Q

What is sickle cell disease?

A

A genetic blood disorder characterized by abnormal hemoglobin that causes red blood cells to deform into a sickle shape.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

True or False: Sickle cell disease is inherited in an autosomal dominant pattern.

A

False

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What type of hemoglobin is produced in sickle cell disease?

A

Hemoglobin S (HbS)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Fill in the blank: Individuals with sickle cell disease often experience ______ due to decreased blood flow.

A

pain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are common symptoms of sickle cell disease?

A

Anemia, pain crises, swelling of hands and feet, frequent infections.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the most common complication of sickle cell disease?

A

Acute chest syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is the life expectancy for individuals with sickle cell disease compared to the general population?

A

Generally lower than the general population, but improving with treatment.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

True or False: Sickle cell disease can be diagnosed with a blood test.

A

True

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is the primary treatment for sickle cell disease?

A

Pain management and hydroxyurea to reduce the frequency of pain crises.
Sickle cell patients should receive the pneumococcal polysaccharide vaccine every 5 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Fill in the blank: Sickle cell disease is most prevalent in ______ populations.

A

African and Mediterranean

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is the role of hydroxyurea in the treatment of sickle cell disease?

A

It increases fetal hemoglobin production, which helps reduce sickling of red blood cells.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Multiple choice: Which of the following is NOT a treatment for sickle cell disease?

A

C) Antibiotics

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is the significance of fetal hemoglobin in sickle cell disease?

A

Higher levels of fetal hemoglobin can reduce sickling of red blood cells.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is a potential cure for sickle cell disease?

A

Bone marrow or stem cell transplant.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

True or False: Sickle cell disease can lead to organ damage over time.

A

True

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is the genetic mutation responsible for sickle cell disease?

A

A single nucleotide substitution in the HBB gene on chromosome 11.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Fill in the blank: A person with one sickle cell gene and one normal gene is said to be a ______.

A

carrier

18
Q

What are vaso-occlusive crises?

A

Painful episodes caused by sickle-shaped cells blocking blood flow in small vessels.

19
Q

Multiple choice: Which organ is particularly at risk in sickle cell disease?

A

B) Spleen

20
Q

What is the main cause of anemia in sickle cell disease?

A

Destruction of sickle-shaped red blood cells.

21
Q

True or False: Sickle cell disease can affect cognitive function.

A

True

22
Q

What role do vaccinations play in managing sickle cell disease?

A

They help prevent infections due to splenic dysfunction.

23
Q

Fill in the blank: The sickle cell trait is considered ______ and usually does not cause symptoms.

A

asymptomatic

24
Q

What is the importance of regular health check-ups for individuals with sickle cell disease?

A

To monitor for complications and manage symptoms effectively.

25
Q

What is a common complication of sickle cell disease?

A

Stroke

26
Q

What lifestyle changes can benefit individuals with sickle cell disease?

A

Staying hydrated, avoiding extreme temperatures, and managing stress.

27
Q

What laboratory technique is used to diagnose neonates as having HbSS rather than HbA?

A

Haemoglobin electrophoresis

28
Q

Name triggers of a sickle cell crisis?

A

Cold envirnment
Hypoxia
Dehydration
Infection

29
Q

Name the different types of sickle cell crisis:

A

A number of types of crises are recognised:

Thrombotic, ‘vaso-occlusive’, ‘painful crises’
Acute chest syndrome
Anaemic
Aplastic
Sequestration
Infection

30
Q

What is the presentation of acute chest syndrome?

A

dyspnoea, chest pain, pulmonary infiltrates on chest x-ray, low pO2

31
Q

What is the management of acute chest syndrome?

A

pain relief

respiratory support e.g. oxygen therapy

antibiotics: infection may precipitate acute chest syndrome and the clinical findings (respiratory symptoms with pulmonary infiltrates) can be difficult to distinguish from pneumonia

transfusion: improves oxygenation

32
Q

What is aplastic crisis?

A

caused by infection with parvovirus
sudden fall in haemoglobin
bone marrow suppression causes a reduced reticulocyte count

33
Q

What is sequestration crises?

A

sickling within organs such as the spleen or lungs causes pooling of blood with worsening of the anaemia
associated with an increased reticulocyte count

34
Q

What is thrombotic crisis also known as?

A

painful crises or vaso-occlusive crises

35
Q

What is the management of sickle cell crisis?

A

General management
- analgesia e.g. opiates
- rehydrate
- oxygen
- consider antibiotics if evidence of infection
blood transfusion
indications include: severe or symptomatic anaemia, pregnancy, pre-operative
do not rapidly reduce the percentage of Hb S containing cells
- exchange transfusion
indications include: acute vaso-occlusive crisis (stroke, acute chest syndrome, multiorgan failure, splenic sequestration crisis
rapidly reduce the percentage of Hb S containing cells

36
Q

What immediate treatment should a patient with sickle cell crisis recieve?

A

SC morphine within 30 minutes of arrival to the ED as simple analgesia is not effective

If after this first dose they are still in severe pain then you should repeat SC morphine bolus.

37
Q

What are some supportive measures that should be done in sickle cell crisis?

A

Keeping the patient warm
Adequate hydration
Treat for suspected sepsis

38
Q

Where can you find information about a patients target INR range and current schedule?

A

Their Yellow Book

39
Q

How do you manage warfarin perioperatively?

A

Warfarin bridging plan

40
Q

Explain bridging of warfarin:

A

5 day pre op stop warfarin and switch to therapeutic LMWH
Post op restart warfarin alongside prophylactic LMWH until there are no sign of bleeding and then therapeutic LMWH until INR is in target range

41
Q

How is sickle cell anaemia diagnosed?

A

Haemoglobin electrophoresis

GP Extras (5 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions