Brainscape's Knowledge GenomeTM

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


See full index

Haematology Week 3 > Pancytopenia > Flashcards

Pancytopenia Flashcards

1
Q

What is pancytopenia?

A

A deficiency of blood cells of all lineages

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

What cell types are generally included in pancytopenia? Which is excluded?

A

Erythrocytes, platelets and neutrophils / lymphocytes

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

What are the two broad causes of pancytopenia?

A

Reduced production of cells or increased destruction of cells

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

What is the general cause of reduced production of cells leading to pancytopenia?

A

Bone marrow failure

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

Inherited marrow failure syndromes arise due to defects in what?

A

DNA repair and ribosomes

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

What are the 3 main features of inherited marrow failure syndromes?

A

Cancer predisposition, congenital anomalies, impaired haemopoiesis

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

What is the main example of an inherited bone marrow failure syndrome?

A

Fanconi’s anaemia

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

What are some features of Fanconi’s anaemia?

A

Short stature, skin pigment abnormalities, endocrinopathies, CV/GI/renal/haematological abnormalities

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

What is the median age of onset of Fanconi’s anaemia?

A

7 years

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

What will happen to the cell size in Fanconi’s anaemia?

A

Macrocytic

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

Fanconi’s anaemia increases the risk of developing what cancer by more than 50% at 40 years?

A

Leukaemia

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

What are the 3 main groups of acquired primary bone marrow failure?

A

Idiopathic aplastic anaemia, myelodysplastic syndromes and acute leukaemia

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

What is idiopathic aplastic anaemia?

A

An autoimmune attack against haemopoietic stem cells

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

What happens in idiopathic aplastic anaemia?

A

Autoreactive T cells produce cytokines which damage the stem cells, particularly of the myeloid lineage

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

Describe what happens in myelodysplastic syndromes?

A

There is dysplasia (disordered development of cells) with hypercellular marrow. There is then increased apoptosis of progenitor and mature cells

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

Myelodysplastic syndromes most commonly occur in who? They have a propensity for evolution into what?

A

Adults aged > 60 / acute myeloid leukaemia

17
Q

What happens to the cell number in myelodysplastic syndrome? Explain this?

A

They stay the same - though the marrow is hypercellular, there is also increased apoptosis

18
Q

How does acute leukaemia cause pancytopenia?

A

Proliferation of abnormal ‘blast’ cells prevent normal haemopoiesis from occurring

19
Q

Which cell count can be variable in acute leukaemia?

A

WCC

20
Q

What are some examples of acquired secondary bone marrow failure?

A

Drugs, B12/folate deficiency, infiltrative disease, viral infections, storage diseases

21
Q

What are some examples of drugs which can cause pancytopenia?

A

Chemotherapy, chloramphenicol and alcohol

22
Q

What is the main cause of pancytopenia due to increased destruction?

A

Hypersplenism

23
Q

Why does hypersplenism result in pancytopenia?

A

Macrophages in the red pulp phagocytose cells, and there is an increased splenic pool

24
Q

What are some causes of hypersplenism?

A

Congestion (e.g. portal hypertension), systemic disease (e.g. RA - Felty’s disease) or haematological disease (e.g. splenic lymphoma)

25
Q

What is the only cause of pancytopenia which will be hypocellular?

A

Aplastic anaemia

26
Q

What are some causes of pancytopenia which will be hypercellular?

A

Myelodysplastic syndromes, B12/folate deficiency, hypersplenism

27
Q

The clinical features of pancytopenia can reflect what?

A

The lack of circulating blood cells or the underlying cause

28
Q

What are some features of anaemia that can be seen in pancytopenia?

A

Fatigue, SOB, CV collapse in older people

29
Q

What are some features of neutropenia which can be seen in pancytopenia?

A

Infections, usually gram - bacterial or fungal

30
Q

What are some features of thrombocytopenia that can be seen in pancytopenia?

A

Bleeding e.g. bruising, purpura, petichiae

31
Q

After history and exam, what are the first investigations for pancytopenia? What are some additional routine tests which could be guided by these results?

A

FBC and blood film / B12/folate levels, LFTs, virology, autoatibodies

32
Q

If there are no results of the cause of pancytopenia from morphology and routine tests, what is the next step in management?

A

Bone marrow exam

33
Q

How is Fanconi’s anaemia tested for?

A

Cytogenetics - chromosome fragility testing

34
Q

What are some supportive treatments for pancytopenia?

A

Red cell and platelet transfusions / antibiotic prophylaxis/treatment

35
Q

What is the management of a neutropenic fever?

A

Empirical antibiotics without waiting for microbiology results

36
Q

How would the following primary bone marrow disorders be treated: a) malignancy? b) congenital problem? c) idiopathic aplastic anaemia?

A

a) chemotherapy b) stem cell transplant c) immunosuppression

37
Q

How would the following secondary bone marrow disorders be treated: a) drug reaction? b) viral infection? c) B12/folate deficiency?

A

a) stop the drug b) treat viral infection c) replace deficiency

38
Q

What is the treatment of hypersplenism?

A

Treat cause if possible, consider splenectomy

Haematology Week 3 (9 decks)

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