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Flashcards in Pancytopenia Deck (20)
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1
Q

what is pancytopenia?

A

deficiency of blood cells of all lineages

2
Q

causes of pancytopenia

A
  1. reduced production

2. increased destruction

3
Q

reduced production cause of pancytopenia

A

bone marrow failure

4
Q

primary acquired causes of bone marrow failure

A

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

5
Q

secondary acquired causes of bone marrow failure

A

drugs
vitamin deficiency
infiltration= lymphoma, mets
miscellaneous

6
Q

drug causes of bone marrow failure

A
chemotherapy
alcohol
azathioprine
methotrexate
chloramphenicol
7
Q

vitamin deficiency causes of bone marrow failure

A

vitamin B12 and folate affect nuclear maturation of all lineages

8
Q

miscellaneous causes of bone marrow failure

A

storage disorders

viral (HIV)

9
Q

inherited cause of bone marrow failure

A

Fanconi’s anaemia

10
Q

increased destruction cause of pancytopenia

A

hypersplenism

11
Q

causes of hypersplenism

A

splenic congestion (portal hypertension)
RA
splenic lymphoma

12
Q

presentation of pancytopenia

A
anaemia= fatigue, SOB
neutropenia= gram -ve sepsis, fungal
thrombocytopenia= bleeding
13
Q

diagnosis of pancytopenia

A
FBC
blood film
B12/ folate
LFTs
virology, Ab
bone marrow
cytogenetics (chromosome fragility testing in Fanconi's anaemia)
14
Q

management of pancytopenia

A

red cells, platelets
antibiotic prophylaxis
prompt treatment of neutropenic sepsis

15
Q

what is Fanconi’s anaemia?

A

inability to correct strand cross-link damage in DNA

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

16
Q

presentation of Fanconi’s anaemia

A
short stature
skin pigment abnormalities (cafe au lait spots)
skeletal abnormalities
endocrine, hypo-genitalia
median age= 7 years
17
Q

what is aplastic anaemia?

A

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

18
Q

management of aplastic anaemia

A

immunosuppression

19
Q

what is myelodysplastic syndrome (MDS)?

A

dysplasia and hypercellular bone marrow with increased apoptosis of cells

ineffective haemopoiesis

20
Q

what can MDS turn into?

A

AML