Haem- other

Question 1

pancytopenia

Answer 1

a deficiency of blood cells of all lineages (generally excludes lymphocytes)

Question 2

is pancytopenia a diagnosis?

Answer 2

NO- reflects a diagnosis

Question 3

causes of pancytopenia

Answer 3

reduced production
OR
increase destruction

Question 4

causes of reduced production of blood cells

Answer 4

bone marrow failure- acquired/inherited/secondary

Question 5

Inherited marrow failure syndrome- Fanconi’s anaemia CF

Answer 5

short stature
skin pigment abnormalities
hypogenitillia
endocrinopathies
GI
CVS
renal
haematological

Question 6

skin change in Fanconi’s anaemia

Answer 6

cafe au lait spots- flat, light to dark brown patches on the skin

Question 7

idiopathic aplastic anaemia

Answer 7

autoimmune attack against haemopoioetic stem cells

Question 8

myelodysplastic syndromes

Answer 8

increased apoptosis of progenitor and mature cells, propensity for evolution into AML

Question 9

secondary bone marrow failure- drug induced examples

Answer 9

chemotherapy
alcohol
azathioprine
methotrexate

Question 10

causes of increased destruction of blood cells

Answer 10

hypersplenism

Question 11

causes of hypersplenism

Answer 11

splenic congestion- portal hypertension
systemic diseases- RA
haematological diseases- splenic lymphoma