Pancytopaenia

Question 1

define pancytopaenia

Answer 1

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

Question 2

is pancytopaenia a diagnosis

Answer 2

no

Question 3

does pancytopaenia always mean there is bone marrow failure

Answer 3

no

Question 4

what are the 2 main processes behind pancytopaenia

Answer 4

reduced production

increased destruction

Question 5

what can cause reduced production in pancytopaenia

Answer 5

inherited bone marrow failure syndromes

acquired bone marrow failure

Question 6

example of inherited bone marrow failure syndrome

Answer 6

Fanconi’s anaemia

Question 7

what are the 3 features of inherited bone marrow failure syndrome

Answer 7

abnormal haemopoeisis
cancer predisposition
congenital anomalies

Question 8

list causes of primary acquired bone marrow failure

Answer 8

Idiopathic aplastic anaemia
Myelodysplastic syndromes
Acute leukaemia

Question 9

what is idiopathic aplastic anaemia

Answer 9

autoimmune condition attaking HSCs resulting in deficiency of mature blood cells

Question 10

features of myelodysplastic syndromes

Answer 10

dysplasia - disordered development

ineffective haemopoeisis

Question 11

how can acute leukaemia result in acquired bone marrow failure

Answer 11

Leukaemia stem cells proliferate to form abnormal ‘blasts’ which do not differentiate into mature blood cells meaning there is a deficiency in these cells

Question 12

list causes of secondary acquired bone marrow failure

Answer 12

Drugs 
B12/folate deficiency 
Infection 
Infiltration 
HIV, storage diseases

Question 13

which drugs can cause bone marrow failure

Answer 13

chemotherapy
chloramphenicol
alcohol

Question 14

B12 and folate deficiencies result in abnormal nuclear maturation in ALL lineages, true or false

Answer 14

TRUE

Question 15

what results in increased destruction as a cause of pancytopaenia

Answer 15

hypersplenism

Question 16

what is meant by increased splenic pool

Answer 16

there is an increased splenic red cell mass and platelet pol
slow transit time for red cells

Question 17

an enlarged spleen means hypersplenism, true or false

Answer 17

false, may not always correlate

Question 18

what are clinical features of pancytopaenia

Answer 18

anaemia: fatigue, pallor, SOB, CVS compromise
neutropaenia: infections
thrombocytopaenia: bleeding and bruising

Question 19

Investigations for pancytopaenia

Answer 19

History and examination 
FBC
Blood film 
(U+E, LFT, serology, virology, B12, folate)
Bone marrow/Trephine biopsy 
(cytogenetics)

Question 20

Aplastic anaemia causes bone marrow HYPO/HYPERcelllularity

Answer 20

HYPOcellular

Question 21

Do the following cause HYPO/HYPERcellular bone marrow:
Myelodysplastic syndromes
B12 and folate deficiencies
Hypersplenism

Answer 21

HYPERcellular

Question 22

how can you treat pancytopaenia supportively

Answer 22

red cell transfusion
platelet transfusion
(neutrophil transfusion)
prophylactic antibiotics/antifungals

Question 23

in a patient with neutropaenic fever, should you wait for the microbiology results before giving antibiotics

Answer 23

no

give them ASAP

Question 24

How do you treat pancytopaenia specifically

Answer 24

identify and treat the cause
eg drugs - stop them 
malignancy - give chemo 
replace B12 and folate 
etc