-aemias

Question 1

AML risk factors
How common is AML
What is the defining feature

Answer 1

previous chemo / radiotherapy
second most common leukaemia
20% or more blasts in the bone marrow or blood

Question 2

AML presenting symptoms

Answer 2

cytopaenias (symptomatic)
Bone pain
Hyperviscosity
Tumour lysis

Question 3

What are the key AML subtypes

Answer 3

APML
AML with NPM1 mutation
AML with FLT3 mutation

Question 4

AML - favourable genetics

Answer 4

APML (translocation 15:17)
Mutated NPM1

Question 5

AML - unfavourable genetics

Answer 5

17p abberation, mutated TP53, complex karyotype

Question 6

What is the philadelphia chromosome?
What is it associated with?

Answer 6

translocation between Ch 9 and 21
Associated with CML

Question 7

APML survival

Answer 7

95%

Question 8

What does a blood film show for APML

Answer 8

Auer rods and bi-lobed nuclei

Question 9

What is the common treatment for favourable risk AML?

Answer 9

Fit and <70;
induction with 7+3
consolidation with HiDAC for 2-4 cycles

Question 10

What is the common treatment for intermediate risk AML?

Answer 10

fit and <70;
induction with 7+3, to induce remission (bone marrow blasts <5%)
consolidation with HiDAC (then transplant)

Question 11

What is “7+3”?

Answer 11

7 days of cytarabine and 3 days of daunorubicin / idarubicin
given as an inpatient

Question 12

What is HiDAC?

Answer 12

high dose cytarabine

Question 13

What is the gene fusion product in APML?

Answer 13

PML-RARA

Question 14

What is the treatment for APML?

Answer 14

ATRA + Arsenic
associated with fever, oedema, pulmonary infiltrates, serositis
ATRA causes elevated Intracranial pressure

Question 15

What drug targets FLT3?

Answer 15

Midostaurin

Question 16

What adverse event is venetoclax associated with?>

Answer 16

tumour lysis syndrome

Question 17

What is Gemtuzumab ozogamzin?

Answer 17

MAB-cytotoxic conjugate, binds CD33+

Question 18

Diagnosis of MDS

Answer 18

Bone marrow suggestive of MDS plus at least one cytopaenia

Question 19

Treatment of MDS

Answer 19

Depends on severity
Use azacitidine (hypomethylating agent)

Question 20

Azacitidine adverse effects

Answer 20

usually well tolerated, minimal nausea, infection in 20%

Question 21

CML common mutation / gene product

Answer 21

Philadelphia chromosome (t(9:22)) fusion gene is BCR:ABL1

Question 22

Phases of CML

Answer 22

Chronic phase (neutrophilia and myelocytosis in peripheral blood)
Accelerated phase (increased blasts in BM, increasing splenomegaly)
Blast phase (acute leukaemia)

Question 23

CML diagnosis

Answer 23

PCR on peripheral blood to confirm translocation
Bone marrow biopsy to assess phase

Question 24

CML treatment

Answer 24

TKI
1st gen - imatinib
2nd gen - dasatinib
3rd gen - ponatinib
side effects; rash, GIT

Question 25

Types of MPN

Answer 25

Essential thrombocythaemia
Polcythaemia vera
Primary myelofibrosis

Question 26

Diagnosis of essential thrombocythaemia

Answer 26

plt >450
BMBx; megakaryocyte proliferation
presence of JAK2, CALR or MPL

Question 27

Treatment of ET

Answer 27

depends on risk
higher risk if evidence of thrombosis or JAK mutation or cardiovascular disease
low risk give aspirin OD or BD
intermediate risk give aspirin plus cytoreduction
high risk give cytoreduction and anticoagulant / aspirin

Question 28

Polycythaemia vera symptoms

Answer 28

fatigue, aquagenic pruritis, headache, splenomegaly, thrombosis

Question 29

Polycythaemia vera diagnosis

Answer 29

Hb>165 (160 in Females)
Hypercellularity in bone marrow
Presence of JAK2 mutation

Question 30

Polycythaemia vera treatment

Answer 30

venesect all patient to hematocrit of <0.45 and daily aspirin
Low risk give BD aspirin
High risk give BD aspirin or anticoagulation

Question 31

Primary myelofibrosis features

Answer 31

megakaryocyte proliferation and bone marrow fibrosis with extramedullary haematopoiesis
Hepatosplenomegaly common, symptomatic anaemia
Sometimes gout or renal stones associated with hyperuricaemia

Question 32

Myelofibrosis diagnosis

Answer 32

Megakaryocytes and fibrosis
presence of JAK2, CALR, MPL plus minor criteria