What is pancytopaenia?
Deficency of blood cells of all lineages
Is lymphopaenia typically a part of pancytopaenia?
Is pancytopaenia a diagnosis?
Sign of underlying problem
Pancytopaenia is either a problem with increased ___ or decreased ___.
What is the underlying reason for pancytopaenia (decreased production)?
Bone marrow failure
Bone marrow failure can be ___ or acquired.
Name an inherited disease causing pancytopaenia.
What are some features of Fanconi anaemia?
Pile of congenital abnormalities
Inherited bone marrow failure is very rare.
What are some accompanying features which would make you suspect an inherited cause of bone marrow failure?
Increased incidence of cancer at a young age
What is the underlying cause of bone marrow failure in inherited syndromes?
Failure of DNA repair
What is idiopathic aplastic anaemia?
Autoimmune destruction of HSCs
Name three causes of a PRIMARY pancytopaenia.
Idiopathic anaplastic anaemia
What happens to the bone marrow in aplastic anaemia?
Widespread replacement of red marrow with yellow marrow
Because there are no RBCs
What is myelodysplasia?
Abnormal growth of bone marrow
Why does myelodysplasia cause primary bone marrow failure?
Cells are crap and die prematurely
What can myelodysplasia progress to?
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
What are some causes of secondary bone marrow failure?
Drug-induced (including alcohol)
B12 / Folate deficiency (accompanying ineffective erythropoiesis)
What happens to any cell which remains in the spleen for too long?
If there is an accumulation of cells in the spleen, what happens?
Increased destruction of blood cells
What are some causes of splenic congestion causing increased destruction of blood cells?
Apart from splenic congestion, what else can cause hypersplenism?
What are the three clinical features of pancytopaenia?
Increased infection risk
What investigations are carried out for patients presenting with pancytopaenia?
other tests depending on these results
In aplastic anaemia, the bone marrow is (hypercellular / hypocellular).
In most causes of pancytopaenia, the bone marrow is (hypercellular / hypocellular).
How is the
b) abnormal bleeding
c) increased infection risk
of pancytopaenia managed?
a) Blood transfusion
b) Platelet transfusion
c) Prophylactic antibiotics
If a patient is neutropaenic and develops a fever, what should you do?
Immediate empirical antibiotics
Don't wait for blood results
How would you treat a pancytopaenia caused by malignancy?
How would you treat a pancytopaenia caused by congenital disease?
Bone marrow transplant