5: Pancytopaenia

Question 1

What is pancytopaenia?

Answer 1

Deficency of blood cells of all lineages

Question 2

Is lymphopaenia typically a part of pancytopaenia?

Answer 2

No

Question 3

Is pancytopaenia a diagnosis?

Answer 3

No

Sign of underlying problem

Question 4

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

Answer 4

increased destruction

decreased production

Question 5

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

Answer 5

Bone marrow failure

Question 6

Bone marrow failure can be ___ or acquired.

Answer 6

inherited

Question 7

Name an inherited disease causing pancytopaenia.

Answer 7

Fanconi anaemia

Question 8

What are some features of Fanconi anaemia?

Answer 8

Pancytopaenia

Pile of congenital abnormalities

Question 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?

Answer 9

Congenital abnormalities

Increased incidence of cancer at a young age

Question 10

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

Answer 10

Failure of DNA repair

Question 11

What is idiopathic aplastic anaemia?

Answer 11

Autoimmune destruction of HSCs

Question 12

Name three causes of a PRIMARY pancytopaenia.

Answer 12

Acute leukaemia

Idiopathic anaplastic anaemia

Myelodysplasia

Question 13

What happens to the bone marrow in aplastic anaemia?

Answer 13

Widespread replacement of red marrow with yellow marrow

Because there are no RBCs

Question 14

What is myelodysplasia?

Answer 14

Abnormal growth of bone marrow

Question 15

Why does myelodysplasia cause primary bone marrow failure?

Answer 15

Cells are crap and die prematurely

‘ineffective erythropoiesis’

Question 16

What can myelodysplasia progress to?

Answer 16

AML

Question 17

Why does acute leukaemia cause primary bone marrow failure?

Answer 17

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

Question 18

What are some causes of secondary bone marrow failure?

Answer 18

Drug-induced (including alcohol)

B12 / Folate deficiency (accompanying ineffective erythropoiesis)

Malignancy

HIV

Question 19

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

Answer 19

Phagocytosis

Question 20

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

Answer 20

Increased destruction of blood cells

=> Hypersplenism

=> Splenomegaly

Question 21

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

Answer 21

Portal hypertension

CCF

Question 22

Apart from splenic congestion, what else can cause hypersplenism?

Answer 22

Inflammatory disease

Malignancy

Question 23

What are the three clinical features of pancytopaenia?

Answer 23

Anaemic symptoms

Abnormal bleeding

Increased infection risk

Question 24

What investigations are carried out for patients presenting with pancytopaenia?

Answer 24

FBC

Blood film

Serum B12/folate

other tests depending on these results

Question 25

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

Answer 25

**hypocellular**

Question 26

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

Answer 26

**hypercellular**

Question 27

How is the a) **anaemia** b) **abnormal bleeding** c) **increased infection risk** of pancytopaenia managed?

Answer 27

**a) Blood transfusion** **b) Platelet transfusion** **c) Prophylactic antibiotics**

Question 28

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

Answer 28

**Immediate empirical antibiotics** Don't wait for blood results

Question 29

How would you treat a **pancytopaenia** caused by **malignancy?**

Answer 29

**Chemotherapy**

Question 30

How would you treat a **pancytopaenia** caused by **congenital disease**?

Answer 30

**Bone marrow transplant**

Question 31

How would you treat a **pancytopaenia** caused by **aplastic anaemia**?

Answer 31

**Immunosuppression**

Question 32

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

Answer 32

**B12 THEN FOLATE SUPPLEMENTS**

Question 33

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

Answer 33

**Withdraw the drug**

Question 34

How would you treat a **pancytopaenia** caused by **hypersplenism**?

Answer 34

**Splenectomy**