Ha - Acute Leukaemia

Question 1

3 year old girl has splenomegaly, lymphadenopathy, low platelets, high WCC. Most likely diagnosis?

Answer 1

ALL

Question 2

define acute leukaemia

Answer 2

neoplastic condition characterised by rapid onset, high mortality if untreated, blast cells and bone marrow failure

Question 3

3 types of bone marrow failure and the sx they manifest

Answer 3

anaemia - pallor, SoB, fatigue
neutropenia - infections
thrombocytopaenia - bleeding

Question 4

name monocyte cells

Answer 4

neutrophils
eosinophils

Question 5

which cell gives rise to AML

Answer 5

multipotent myeloid stem cell or pluripotent stem cell

Question 6

which cell gives rise to ALL

Answer 6

common lymphoid stem cell

Question 7

what cell type is dominant in acute leukaemias

Answer 7

BLAST CELLS

Question 8

granules on a cell indicate what

Answer 8

myeloid cell

Question 9

age trend in AML

Answer 9

increased with age, except blip in first year of life

Question 10

median age of AML

Answer 10

65-70

Question 11

how does age affect prognosis of AML q

Answer 11

prognosis worse with icnreasing age

Question 12

what % of adults cured of AML

Answer 12

40%

Question 13

what causes AML

Answer 13

abberations in chromosome count / structure –> eg translocations, trisomy

Question 14

which type of acute leukaemia gets abnormal regulation of genes

Answer 14

ALL

Question 15

which leukaemia has creation of new genes involved

Answer 15

ALL and AML

Question 16

is chromosomal duplication common in AML

Answer 16

yes

Question 17

which chromosomes are most likely to be duplicated to cause AML

Answer 17

8 and 21

Question 18

is chromosome loss / deletion common in AML

Answer 18

yes

Question 19

which chromosomes are deleted / lost in AML

Answer 19

5 and 7

Question 20

how does chromosome deletion / duplication cause AML

Answer 20

deletion - loss of TSG
duplication - more oncogenes

Question 21

what type of molecular abnormalities can be seen in normal chromosomes in acute leukaemia

Answer 21

point mutations
loss of TSG
partial duplication
cryptic deletion

Question 22

describe the cell development process that leads to AML formation

Answer 22

block in maturation of myelocyte cells so blast cells accumulate

Question 23

why do people get AML

Answer 23

familial / syndromic predisposition
irridation
anti cancer drugs
smoker
unknown !

Question 24

how many molecular insults are needed to cause leukaemia

Answer 24

at least 2

Question 25

what are type 1 abnormalities in AML

Answer 25

promote proliferation and survival

Question 26

what are type 2 abnormalities in AML

Answer 26

block differentiation (which normally preceeds apoptosis)

Question 27

for which specific type of AML is the mechanism understood and a cure available to patients

Answer 27

t(15;17)

Question 28

complication of t(15;17) AML

Answer 28

DIC

Question 29

what genes are fused in t(15;17) AML

Answer 29

PML and RARA

Question 30

is transcription factor disturbance enough to cause AML

Answer 30

no - need further genetic hit

Question 31

what cells have auer rods

Answer 31

myeloid cells

Question 32

what confirms AML vs ALL

Answer 32

immunophenotyping

Question 33

which stains are positive in AML but negative in ALL

Answer 33

myeloperoxidase sudan black non specific esterase

Question 34

how does immunophenotyping work

Answer 34

AB recognises specific antigen. AB is then flagged with a immunoflurescent dye to recognise it

Question 35

Sx of AML

Answer 35

anaemia, neutropenia, thrombocytopaenia hepatosplenomegaly lymphadenopathy / gum infiltration

Question 36

which type of AML has gum infiltration

Answer 36

acute monocytic leukaemia

Question 37

which type of AML gets CNS disease (eye palsy etc) more commonly than others

Answer 37

acute monocytic also ALL

Question 38

what can bleeding in AML lead to

Answer 38

DIC

Question 39

what eye signs can be seen due to AML and why

Answer 39

papilloedema / retinal haemorrhages due to hyper-viscosity of blood

Question 40

how is AML diagnosed

Answer 40

blood film - diagnostic usually

Question 41

what is seen on blood film to diagnose AML

Answer 41

blasts / auer rods

Question 42

leukaemia Sx. No abnormal cells on film. What do you do?

Answer 42

bone marrow aspirate

Question 43

leukaemia Sx. No abnormal cells on film. likely dx?

Answer 43

aleukaemic leukaemia - blasts confined to bone marrow so cant see on film

Question 44

why are cytogenetoc / molecular studies done on AML pts?

Answer 44

prognostic value selective tx targets

Question 45

which gene patterns convey the best prognosis of AML

Answer 45

t(15;17) t(8;21) inv(16) or t(16;16)

Question 46

which gene patterns convey the worst prognosis of AML

Answer 46

-5, del 5 -7, 3q- complex

Question 47

supportive care Tx of AML

Answer 47

red cells platelets FFP Abx long line allopurinol

Question 48

mx of AML

Answer 48

supportive care chemo targeted molecular therapy transplant

Question 49

describe chemo regime of AML

Answer 49

cell cycle specific drugs 4-5 courses over 6 months - remission induction x2 - consolidation x2 or 3

Question 50

give some examples of molecular therapies for acute promyelocytic leukaemia

Answer 50

all trans retinoic acid (ATRA) arsenic trioxide

Question 51

molecular therapies for acute Ph+ leukaemia

Answer 51

tyrosine kinase inhibitors

Question 52

antibody tx for AML

Answer 52

gemtuzumab ozogamicin

Question 53

what determines prognosis of AML

Answer 53

pt charactristics morphology / immunophenotyping response to tx