5: Pancytopaenia Flashcards
What is pancytopaenia?
Deficency of blood cells of all lineages
Is lymphopaenia typically a part of pancytopaenia?
No
Is pancytopaenia a diagnosis?
No
Sign of underlying problem
Pancytopaenia is either a problem with increased ___ or decreased ___.
increased destruction
decreased production
What is the underlying reason for pancytopaenia (decreased production)?
Bone marrow failure
Bone marrow failure can be ___ or acquired.
inherited
Name an inherited disease causing pancytopaenia.
Fanconi anaemia
What are some features of Fanconi anaemia?
Pancytopaenia
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?
Congenital abnormalities
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.
Acute leukaemia
Idiopathic anaplastic anaemia
Myelodysplasia
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
‘ineffective erythropoiesis’
What can myelodysplasia progress to?
AML
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)
Malignancy
HIV
What happens to any cell which remains in the spleen for too long?
Phagocytosis
If there is an accumulation of cells in the spleen, what happens?
Increased destruction of blood cells
=> Hypersplenism
=> Splenomegaly
What are some causes of splenic congestion causing increased destruction of blood cells?
Portal hypertension
CCF
Apart from splenic congestion, what else can cause hypersplenism?
Inflammatory disease
Malignancy
What are the three clinical features of pancytopaenia?
Anaemic symptoms
Abnormal bleeding
Increased infection risk
What investigations are carried out for patients presenting with pancytopaenia?
FBC
Blood film
Serum B12/folate
other tests depending on these results
In aplastic anaemia, the bone marrow is (hypercellular / hypocellular).
hypocellular
In most causes of pancytopaenia, the bone marrow is (hypercellular / hypocellular).
hypercellular
How is the
a) anaemia
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?
Chemotherapy
How would you treat a pancytopaenia caused by congenital disease?
Bone marrow transplant
How would you treat a pancytopaenia caused by aplastic anaemia?
Immunosuppression
How would you treat a pancytopaenia caused by B12 / folate deficiency?
B12 THEN FOLATE SUPPLEMENTS
How would you treat a pancytopaenia caused by a drug reaction?
Withdraw the drug
How would you treat a pancytopaenia caused by hypersplenism?
Splenectomy
