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Haematology Week 3 2018/19 > 5: Pancytopaenia > Flashcards

5: Pancytopaenia Flashcards

1
Q

What is pancytopaenia?

A

Deficency of blood cells of all lineages

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2
Q

Is lymphopaenia typically a part of pancytopaenia?

A

No

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3
Q

Is pancytopaenia a diagnosis?

A

No

Sign of underlying problem

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4
Q

Pancytopaenia is either a problem with increased ___ or decreased ___.

A

increased destruction

decreased production

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5
Q

What is the underlying reason for pancytopaenia (decreased production)?

A

Bone marrow failure

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6
Q

Bone marrow failure can be ___ or acquired.

A

inherited

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7
Q

Name an inherited disease causing pancytopaenia.

A

Fanconi anaemia

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8
Q

What are some features of Fanconi anaemia?

A

Pancytopaenia

Pile of congenital abnormalities

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9
Q

Inherited bone marrow failure is very rare.

What are some accompanying features which would make you suspect an inherited cause of bone marrow failure?

A

Congenital abnormalities

Increased incidence of cancer at a young age

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10
Q

What is the underlying cause of bone marrow failure in inherited syndromes?

A

Failure of DNA repair

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11
Q

What is idiopathic aplastic anaemia?

A

Autoimmune destruction of HSCs

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12
Q

Name three causes of a PRIMARY pancytopaenia.

A

Acute leukaemia

Idiopathic anaplastic anaemia

Myelodysplasia

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13
Q

What happens to the bone marrow in aplastic anaemia?

A

Widespread replacement of red marrow with yellow marrow

Because there are no RBCs

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14
Q

What is myelodysplasia?

A

Abnormal growth of bone marrow

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15
Q

Why does myelodysplasia cause primary bone marrow failure?

A

Cells are crap and die prematurely

‘ineffective erythropoiesis’

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16
Q

What can myelodysplasia progress to?

A

AML

17
Q

Why does acute leukaemia cause primary bone marrow failure?

A

Leukaemic stem cells (LSCs) can only proliferate, they don’t differentiate or mature

LSCs then ‘crowd out’ normal HSCs from their niche in the bone marrow, causing a pancytopaenia

18
Q

What are some causes of secondary bone marrow failure?

A

Drug-induced (including alcohol)

B12 / Folate deficiency (accompanying ineffective erythropoiesis)

Malignancy

HIV

19
Q

What happens to any cell which remains in the spleen for too long?

A

Phagocytosis

20
Q

If there is an accumulation of cells in the spleen, what happens?

A

Increased destruction of blood cells

=> Hypersplenism

=> Splenomegaly

21
Q

What are some causes of splenic congestion causing increased destruction of blood cells?

A

Portal hypertension

CCF

22
Q

Apart from splenic congestion, what else can cause hypersplenism?

A

Inflammatory disease

Malignancy

23
Q

What are the three clinical features of pancytopaenia?

A

Anaemic symptoms

Abnormal bleeding

Increased infection risk

24
Q

What investigations are carried out for patients presenting with pancytopaenia?

A

FBC

Blood film

Serum B12/folate

other tests depending on these results

25
Q

In aplastic anaemia, the bone marrow is (hypercellular / hypocellular).

A

hypocellular

26
Q

In most causes of pancytopaenia, the bone marrow is (hypercellular / hypocellular).

A

hypercellular

27
Q

How is the

a) anaemia
b) abnormal bleeding
c) increased infection risk

of pancytopaenia managed?

A

a) Blood transfusion

b) Platelet transfusion

c) Prophylactic antibiotics

28
Q

If a patient is neutropaenic and develops a fever, what should you do?

A

Immediate empirical antibiotics

Don’t wait for blood results

29
Q

How would you treat a pancytopaenia caused by malignancy?

A

Chemotherapy

30
Q

How would you treat a pancytopaenia caused by congenital disease?

A

Bone marrow transplant

31
Q

How would you treat a pancytopaenia caused by aplastic anaemia?

A

Immunosuppression

32
Q

How would you treat a pancytopaenia caused by B12 / folate deficiency?

A

B12 THEN FOLATE SUPPLEMENTS

33
Q

How would you treat a pancytopaenia caused by a drug reaction?

A

Withdraw the drug

34
Q

How would you treat a pancytopaenia caused by hypersplenism?

A

Splenectomy

Haematology Week 3 2018/19 (10 decks)

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