Pancytopenia

Question 1

what is pancytopenia?

Answer 1

deficiency of blood cells of all lineages

Question 2

causes of pancytopenia

Answer 2

1. reduced production

2. increased destruction

Question 3

reduced production cause of pancytopenia

Answer 3

bone marrow failure

Question 4

primary acquired causes of bone marrow failure

Answer 4

aplastic anaemia
MDS
acute leukaemia (proliferation of abnormal cells blocks HSC development)

Question 5

secondary acquired causes of bone marrow failure

Answer 5

drugs
vitamin deficiency
infiltration= lymphoma, mets
miscellaneous

Question 6

drug causes of bone marrow failure

Answer 6

chemotherapy
alcohol
azathioprine
methotrexate
chloramphenicol

Question 7

vitamin deficiency causes of bone marrow failure

Answer 7

vitamin B12 and folate affect nuclear maturation of all lineages

Question 8

miscellaneous causes of bone marrow failure

Answer 8

storage disorders

viral (HIV)

Question 9

inherited cause of bone marrow failure

Answer 9

Fanconi’s anaemia

Question 10

increased destruction cause of pancytopenia

Answer 10

hypersplenism

Question 11

causes of hypersplenism

Answer 11

splenic congestion (portal hypertension)
RA
splenic lymphoma

Question 12

presentation of pancytopenia

Answer 12

anaemia= fatigue, SOB
neutropenia= gram -ve sepsis, fungal
thrombocytopenia= bleeding

Question 13

diagnosis of pancytopenia

Answer 13

FBC
blood film
B12/ folate
LFTs
virology, Ab
bone marrow
cytogenetics (chromosome fragility testing in Fanconi's anaemia)

Question 14

management of pancytopenia

Answer 14

red cells, platelets
antibiotic prophylaxis
prompt treatment of neutropenic sepsis

Question 15

what is Fanconi’s anaemia?

Answer 15

inability to correct strand cross-link damage in DNA

macrocytosis > thrombocytopenia > neutropenia (bone marrow failure and leukaemia risk)

Question 16

presentation of Fanconi’s anaemia

Answer 16

short stature
skin pigment abnormalities (cafe au lait spots)
skeletal abnormalities
endocrine, hypo-genitalia
median age= 7 years

Question 17

what is aplastic anaemia?

Answer 17

AI disorder against HSCs, auto-reactive T cells secrete IFN-gamma and TNF-alpha

Question 18

management of aplastic anaemia

Answer 18

immunosuppression

Question 19

what is myelodysplastic syndrome (MDS)?

Answer 19

dysplasia and hypercellular bone marrow with increased apoptosis of cells

ineffective haemopoiesis

Question 20

what can MDS turn into?

Answer 20

AML