pancytopenia

Question 1

pancytopenia

Answer 1

deficiency of blood cells of ALL lineages - but generally excludes lymphocytes
- not a diagnosis
- not always mean bone marrow falure or malignnacy

Question 2

lifespan of red cells

Answer 2

120days

Question 3

lifespan of neutrophils

Answer 3

7-8hrs

why so many neutrophils are needed cos they die so quick

Question 4

lifespan of platelets

Answer 4

7-10days

-> why aspirin needs stopped 7 days before surgery

Question 5

causes of pancytopenia

Answer 5

reduced production
- bone marrow failure
- inherited conditions

increased destruction -> hypersplenism

Question 6

inherited causes of pancytopenia

Answer 6

fanconi’s anaemia

Question 7

fanconi’s anaemia

Answer 7

unable to correct inter-strand cross-links (DNA damage)
- macrocytosis followed by thrombocytopenia, then neutrophilia

bone marrow failure (aplasia) risk = 84% by 20yrs
leukaemia risk 52% bt 40yrs

median age of presentation = 7

Question 8

fanconi’s anaemia presentation

Answer 8

short stature
skin pigment abnormalities - cafe au lait
skeletal - 4fingers
radial ray abnormalities
hypogenitalia
endocrinopathies
GI defects
CVS, renal, haematological

aplastic anaemia

Question 9

causes of acquired primary bone marrow failure

Answer 9

idiopathic aplastic anaemia
myelodysplastic syndromes
acute leukaemia (total white cell count can be high due to an excess of circulating blasts)

Question 10

idiopathic aplastic anaemia

Answer 10

autoimmune attack against haemopoietic stem cell
- no longer produces mature cells

abnormal population of auto reactive T cells attacking precursor cells

Question 11

myelodysplastic syndromes

Answer 11

dysplasia + excess of apoptosis of progenitor + mature cells in bone marrow (ineffective haemopoiesis)

propensity for evolution into AML

Question 12

why can acute lekaemia cause pancytopenia?

Answer 12

proliferation of abnormal cells (blasts) from leukaemia stem cells which fail to differentiate or mature into normal cells

prevent normal haemopoietic stem/progenitor development by “hijacking/altering” the haemopoietic niche + marrow microenvironment

Question 13

causes of secondary bone marrow failure

Answer 13

drug induced - causes aplasia
- chemo, alcohol
- azathioprine, methotrexate
- chlormaphenicol

B12/folate deficiency (nuclear maturation can affect all lineages)
- hypercellular marrow in these patients

infiltrative - non-haemopoietic malignant infiltration, lymphoma

infections, viral - HIV
storage disorders

Question 14

hypersplenism

Answer 14

increased splenic pool (platelets), increased red cell mass, slower red cell transit

increased destruction that exceeds bone marrow capacity, usually assoc with significantly enlarged spleen

splenic size alone may not always correlate with hypersplenism

Question 15

causes of hypersplenism

Answer 15

congestion - portal hypertension
systemic disease - rheumatoid arthritis

haematological disease - splenic lymphoma

Question 16

test for fanconis

Answer 16

cytogenetics, chromosome fragility testing

Question 17

causes of hypocellular marrow in pancytopenia

Answer 17

aplastic anaemia

Question 18

causes of hypercellular marrow in pancytopenia

Answer 18

myelodysplastic syndromes
B12/folate deficiency
hypersplenism

Question 19

treatment of pancytopenia

Answer 19

supportive
- red cell transfusions
- platelet transfusions
- neutrophil transfusions not routine

antibiotics prophylaxis/treatment
- antibacterials - treat neutropenic fever without waiting for microbiology results
- antifungals

then specific treatment depending on cause

Question 20

treatment of a primary bone marrow disorder causing pancytopenia

Answer 20

malignancy - chemo

congenital - bone marrow transplantation

idiopathic aplastic anaemia - immunosuppression

Question 21

treatment of a secondarybone marrow disorder causing pancytopenia

Answer 21

drug reation - stop drug

viral - treat HIV

replace b12/folate

Question 22

treatment of hypersplensism causing pancytopenia

Answer 22

treat cause if possible
consider splenectomy - not always appropriate