Pancytopenia

Question 1

define pancytopenia

Answer 1

a deficiency of blood cells of all lineages

Question 2

what lineage tends to not be included in pancytopenia

Answer 2

lymphocytes

Question 3

pancytopenia indicates bone marrow failure T or F

Answer 3

F, not always

Question 4

what lineages are low in pancytopenia

Answer 4

erythrocytes
platelets
granulocytes

Question 5

fundamental causes of pancytopenia?

Answer 5

reduced production

increased destruction

Question 6

bone marrow failure is more commonly acquired or inherited

Answer 6

acquired

Question 7

triad of characteristics in inherited bone marrow failure?

Answer 7

cancer predisposition
impaired haemopoeisis
congenital abnormalities

Question 8

give an example of an inherited bone marrow failure

Answer 8

fanconi’s anaemia

Question 9

what bone is malformed or completely lost in fanconi’s anaemia?

Answer 9

radial

Question 10

what cells tend to be lost first in fanconi’s anaemia

Answer 10

macrocytosis first
thrombocytopenia next
neutropenia next

Question 11

risk of leukaemia in fanconis?

Answer 11

1 in 2

Question 12

acquired causes of primary bone marrow failure?

Answer 12

myelodysplastic syndromes
acute leukaemia
idiopathic aplastic anaemia

Question 13

what happens in IAA

Answer 13

autoimmune attack by T cells against haemopoeitic STEM cells

Question 14

what does the marrow look like in aplastic anaemia?

Answer 14

fatty, lacking erythrocytes

Question 15

what does the marrow look like in MDS?

Answer 15

hypercellular

full of dysplastic cells

Question 16

why is the cell count low in MDS if the marrow is hypercellular?

Answer 16

its ineddective erythopoesis as there is increased apoptosis and dysplasia

Question 17

MDS can become what cancer..

Answer 17

AML

Question 18

dysplastic cells in the bone marrow…

Answer 18

MDS

Question 19

why can acute leukaemia cause pancytopenia?

Answer 19

abnormal blasts proliferate from leukaemic stem cells so are unable to differentiate

Question 20

secondary causes of bone marrow failure?

Answer 20

drugs eg chemo/alcohol cause aplasia
B12/folate deficiency
lymohoma/mets
HIV
storage disease

Question 21

B12/folate deficiency causes a ___cellular marrow

Answer 21

hypercellular

inc division in primitive cells

Question 22

drug induced BM failure causes a __cellular marrow

Answer 22

hypo

Question 23

why is it important that cells transitting through the spleen do so quickly?

Answer 23

macrophages within the cords of the spleen will destroy blood cells if theyre slow

Question 24

you have a/an ___ed splenic pool in hypersplenism

Answer 24

increased

Question 25

red cell transit is fast/slow in hypersplenism

Answer 25

slow

Question 26

what haemotological cancer can cause hypersplenism?

Answer 26

splenic lymphoma

Question 27

triad of issues in pancytopenia

Answer 27

anaemia
neutropenia
thrombocytopenia

Question 28

symptoms of anaemia

Answer 28

fatigue
SoB
cardiovascular comprise

Question 29

symptoms of neutropenia

Answer 29

infection esp gram -ve

Question 30

symptoms of thrombocytopenia

Answer 30

purpura
petechiae
bleeds

Question 31

Ix pancytopenia

Answer 31

1. and film
2. based on findings:
   B12/folate
   LFTs
   virology
   autoantibody
   cytogenics
   chromosomal

Question 32

macrovalocytes + hypersegmented neutrophils…

Answer 32

B12/folate deficiency

Question 33

Tx pancytopenia

Answer 33

treat cause

replace losses eg transfusion/antibiotics

Question 34

neutropenic fever should get antibiotics straight away without waiting for microbio results T or F

Answer 34

T

Question 35

Tx malignancy that is causing a primary BM disorder

Answer 35

chemo

Question 36

Tx congenital problem that is causing a primary BM disorder

Answer 36

bone marrow transplant

Question 37

Tx IAA

Answer 37

immunosuppression

Question 38

Tx hypersplenism

Answer 38

treat cause if possible

consider splenectomy