Pancytopenia

Question 1

What is pancytopenia?

Answer 1

A deficiency of blood cells of all lineages

Question 2

What is important to remember about pancytopenia.

Answer 2

It is not a diagnosis!

It does not always mean bone marrow failure

Question 3

What is the life span of red cells?

Answer 3

Around 120 dyas

Question 4

What is the lifespan of neutrophils

Answer 4

7-8 hours

Question 5

What is the lifespan of platelets

Answer 5

7-10 days

Question 6

What causes a pancytopenia

Answer 6

Reduced production or increased destruction

Question 7

What would be the potential causes of a ‘reduced production’ pancytopenia?

Answer 7

Bone marrow failure

• Inherited
• Acquired (primary or secondary)

Question 8

Name an example of an inherited case of marrow failure

Answer 8

Fanconi anaemia

rare

Question 9

What are the clinical features of fanconi anaemia

Answer 9

Short stature
Skin pigment abnormalities - café au lait spots
Radial ray abnormalities
Hypogenitilia
Endocrinopathies
GI defects
Cardiovascular
Renal 
Haematological

Question 10

What haematological abnormalities are present in fanconi anaemia

Answer 10

Unable to correct inter-strand cross-links (DNA damage).

This results in bone marroe failure (aplasia) in 84 percent by age 20.
Leukaemia risk is increased. (52 percent by 40 yrs).

Question 11

What is the median age that haematological abnormalities occur in fanconi anaemia

Answer 11

7 yrs

Question 12

What acquired primary bone marrow failure can occur.

Answer 12

1. Aplastic anaemia
2. Myelodyspastic syndromes
3. Acute leukaemia

Question 13

What is aplastic anaemia>

Answer 13

Autoimmune attack against haemopoietic stem cells.

Question 14

What are myelodysplastic syndromes.

Answer 14

Increased apoptosis of progenitor and mature cells (ineffective haemipoiesis)

Question 15

What are the causes of secondary bone marrow failure?

Answer 15

1. Drug induced
2. B12/folate
3. Malignant - lymphoma, non haemopoietic infiltration
4. Viral (HIV)

Question 16

What drugs can cause aplasia?

Answer 16

Chloramphenicol
Alcohol
Chemotherapy

Question 17

Why can B12/folate deficiency result in marrow failure

Answer 17

they are required for nuclear maturation and can affect all lineages of blood cell.

Question 18

What can cause increased destruction of cells and result in pancytopaenia

Answer 18

Hypersplenism

Autoimmune

Question 19

What is hypersplenism

Answer 19

Increases splenic pool results in increased destruction that exceeds bone marrow capacity. Usually associated with an enlarged spleen.

Question 20

What can cause hypersplenism

Answer 20

Any cause of splenomegaly can potentially result in hypersplenism. (eg thalassaemia, EBV, spherocytosis, sickle cell anaemia, liver cirrhosis).

However splenic size may not always correlate with hypersplenism.

Question 21

What results in splenic congestion resulting in hypersplenism>

Answer 21

Portal hypertension

Congestic cardiac failure

Question 22

What systemic diseases can result in hypersplenism

Answer 22

rheumatoid arthritis

Question 23

What haematological diseases can result in hypersplenism

Answer 23

Myelofibrosis

Question 24

What are the main differences in splenic pool in hypersplenism

Answer 24

Splenic red cell mass - normal = 5 percent, hypersplenism= 40 percent

Red cell transit- normal = fast, hypersplenism= slow

Splenic platelet pool- normal = 20-40 percent, hypersplenism= 90 percent

Question 25

What are the signs that directly relate to pancytopenia?

Answer 25

Anaemia + neutropenia + thrombocytopenia

Question 26

what will the cellularity of the pancytopenia be in aplastic anaemia

Answer 26

Hypocellular

Question 27

When with the bone marrow be hypercellular?

Answer 27

Myelodysplastic syndromes
b12/folate deficiency
Hypersplenism