5: Pancytopaenia Flashcards Preview

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

Flashcards in 5: Pancytopaenia Deck (34)
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1

What is pancytopaenia?

Deficency of blood cells of all lineages

2

Is lymphopaenia typically a part of pancytopaenia?

No

3

Is pancytopaenia a diagnosis?

No

Sign of underlying problem

4

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

increased destruction

decreased production

5

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

Bone marrow failure

6

Bone marrow failure can be ___ or acquired.

inherited

7

Name an inherited disease causing pancytopaenia.

Fanconi anaemia

8

What are some features of Fanconi anaemia?

Pancytopaenia

Pile of congenital abnormalities

9

Inherited bone marrow failure is very rare.

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

Congenital abnormalities

Increased incidence of cancer at a young age

10

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

Failure of DNA repair

11

What is idiopathic aplastic anaemia?

Autoimmune destruction of HSCs

12

Name three causes of a PRIMARY pancytopaenia.

Acute leukaemia

Idiopathic anaplastic anaemia

Myelodysplasia

13

What happens to the bone marrow in aplastic anaemia?

Widespread replacement of red marrow with yellow marrow

Because there are no RBCs

14

What is myelodysplasia?

Abnormal growth of bone marrow

15

Why does myelodysplasia cause primary bone marrow failure?

Cells are crap and die prematurely

'ineffective erythropoiesis'

16

What can myelodysplasia progress to?

AML

17

Why does acute leukaemia cause primary bone marrow failure?

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

What are some causes of secondary bone marrow failure?

Drug-induced (including alcohol)

B12 / Folate deficiency (accompanying ineffective erythropoiesis)

Malignancy

HIV

19

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

Phagocytosis

20

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

Increased destruction of blood cells

=> Hypersplenism

=> Splenomegaly

21

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

Portal hypertension

CCF

22

Apart from splenic congestion, what else can cause hypersplenism?

Inflammatory disease

Malignancy

23

What are the three clinical features of pancytopaenia?

Anaemic symptoms

Abnormal bleeding

Increased infection risk

24

What investigations are carried out for patients presenting with pancytopaenia?

FBC

Blood film

Serum B12/folate

other tests depending on these results

25

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

hypocellular

26

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

hypercellular

27

How is the

a) anaemia

b) abnormal bleeding

c) increased infection risk

of pancytopaenia managed?

a) Blood transfusion

b) Platelet transfusion

c) Prophylactic antibiotics

28

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

Immediate empirical antibiotics

Don't wait for blood results

29

How would you treat a pancytopaenia caused by malignancy?

Chemotherapy

30

How would you treat a pancytopaenia caused by congenital disease?

Bone marrow transplant