Pancytopenia Flashcards
What is pancytopenia?
A deficiency of blood cells of all lineages
What is important to remember about pancytopenia.
It is not a diagnosis!
It does not always mean bone marrow failure
What is the life span of red cells?
Around 120 dyas
What is the lifespan of neutrophils
7-8 hours
What is the lifespan of platelets
7-10 days
What causes a pancytopenia
Reduced production or increased destruction
What would be the potential causes of a ‘reduced production’ pancytopenia?
Bone marrow failure
- Inherited
- Acquired (primary or secondary)
Name an example of an inherited case of marrow failure
Fanconi anaemia
rare
What are the clinical features of fanconi anaemia
Short stature Skin pigment abnormalities - café au lait spots Radial ray abnormalities Hypogenitilia Endocrinopathies GI defects Cardiovascular Renal Haematological
What haematological abnormalities are present in fanconi anaemia
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).
What is the median age that haematological abnormalities occur in fanconi anaemia
7 yrs
What acquired primary bone marrow failure can occur.
- Aplastic anaemia
- Myelodyspastic syndromes
- Acute leukaemia
What is aplastic anaemia>
Autoimmune attack against haemopoietic stem cells.
What are myelodysplastic syndromes.
Increased apoptosis of progenitor and mature cells (ineffective haemipoiesis)
What are the causes of secondary bone marrow failure?
- Drug induced
- B12/folate
- Malignant - lymphoma, non haemopoietic infiltration
- Viral (HIV)
What drugs can cause aplasia?
Chloramphenicol
Alcohol
Chemotherapy
Why can B12/folate deficiency result in marrow failure
they are required for nuclear maturation and can affect all lineages of blood cell.
What can cause increased destruction of cells and result in pancytopaenia
Hypersplenism
Autoimmune
What is hypersplenism
Increases splenic pool results in increased destruction that exceeds bone marrow capacity. Usually associated with an enlarged spleen.
What can cause hypersplenism
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.
What results in splenic congestion resulting in hypersplenism>
Portal hypertension
Congestic cardiac failure
What systemic diseases can result in hypersplenism
rheumatoid arthritis
What haematological diseases can result in hypersplenism
Myelofibrosis
What are the main differences in splenic pool in hypersplenism
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
What are the signs that directly relate to pancytopenia?
Anaemia + neutropenia + thrombocytopenia
what will the cellularity of the pancytopenia be in aplastic anaemia
Hypocellular
When with the bone marrow be hypercellular?
Myelodysplastic syndromes
b12/folate deficiency
Hypersplenism
