Pancytopenia

Question 1

Pancytopenia = decreased cells in all lineages

Answer 1

Not a diagnosis, does not always mean marrow failure.

Question 2

Lifespan of RBC?

Lifespan of neutrophil?

Lifespan of platelet?

Answer 2

RBC = 120 days.

Neutrophils = 7-8 hours.

Platelets = 7-10 days.

Question 3

What is Fanconi’s anaemia?

Answer 3

An autosomal recessive condition associated with failure to repair DNA cross links.

Presents with pancytopenia, short stature, missing thumb/radii, hypogenitalia.

Increased risk of AML,

Median age = 7 years.

Management = stem cell transplant.

Question 4

What is aplastic anaemia?

Answer 4

Autoimmune attack against haemopoietic stem cell.

Question 5

Management of aplastic anaemia?

Answer 5

Identify cause and treat +/- splenectomy.
Supportive treatment
- red cell/platelet transfusion
- infection prophylaxis.

Question 6

What is Felty’s syndrome?

Answer 6

1) Splenomegaly
2) Rheumatoid arthritis
3) Low white cell count.

Question 7

Symptoms and signs of pancytopenia (think about cells)

Answer 7

Anaemia (fatigue, SOB, CVS compromise)

Neutropenia (infections)

Thrombocytopenia (Bleeding, purpura, petechia)