43 Acute Leukaemia

Question 1

How does acute leukaemia arise?

Answer 1

De novo or terminal event of pr-existing blood disorder.

Question 2

Which type of cell is seen in the blood in acute leukaemia?

What is the difference in these cells between AML and ALL?

Answer 2

Blast cells.

Bigger nucleus and more cytoplasm in AML.

Question 3

How does acute leukaemia present? (3).

Answer 3

Anaemia.
Infections.
Easy bruising and haemorrhage.

Question 4

Where might leukaemia cells infiltrate into in acute leukaemia? (5).

Answer 4

Kidney, spleen, meninges, testes and skin.

Rarer to occur outside marrow in AML.

Question 5

Which infections commonly occur in leukaemia? (3,3)

Answer 5

Bacterial: septicaemia, pneumonia, skin sepsis.
Fungal: oral, septicaemia, organ

Question 6

What is acute leukaemia?

Answer 6

Accumulation of early myeloid or lymphoid precursors in the bone marrow, blood or other tissues.

Question 7

What are the two classifications of AML?

Answer 7

FAB.

WHO.

Question 8

How is acute leukaemia diagnosed?

Answer 8

Monoclonal antibody determination of surface antigens using immunofluorescence.

Question 9

Which AML genetic abnormalities have favourable prognosis? (3).

Answer 9

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

Question 10

Which AML genetic abnormalities have unfavourable prognosis? (4).

Answer 10

t(6;9)
inv(3)
del(5q)
-7, -5

Question 11

How does the genetic basis of ALL differ to AML?

Answer 11

ALL is mostly changes in chromosome number.

Question 12

Which chromosomal abnormality has a favourable prognosis in ALL?

Answer 12

High hyperpoloidy, >50 chromosomes.

Question 13

Which chromosomal changes have a very poor prognosis in ALL?

Answer 13

t(8;14)

t (9;22) Philadelphia chromosome.

Question 14

What is the name for the t(9;22) translocation?

Answer 14

Philadelphia chromosome.

Question 15

What fusion product does the t(8;21) translocation result in?

Answer 15

AML-ETO.

Question 16

Which translocation and associated gene product explains the response of promyelocytic leukaemia to all trans retinoid acid (ATRA)?

Answer 16

t(15;17)

PML-RARα

Question 17

Which mutation are present in cytologically normal AML?

Associated prognosis?

Answer 17

NPM1 = good.
FLT3 = bad.

Question 18

What are the poor prognostic features in ALL? (6).

Answer 18

Increasing age.
High WCC.
Male sex. 
Certain cytogenetic abnormalities.
Poor treatment response.
T-ALL or null-ALL

Question 19

How is AML managed?

Answer 19

Induction Rx to obtain remission, then consolidation with further courses of chemo.
Possible bone marrow transplant in young.

Question 20

How is ALL managed?

Answer 20

Induction chemo, intensive consolidation chemo.
Prophylaxis of meningeal leukaemia: methotrexate + cranial irradiation.
Maintenance chemo.
Bone marrow transplant in high risk.

Question 21

What are the complications of therapy in acute leukaemia? (2)

Answer 21

Neutropenic sepsis (all patients).
Neutropenic fever: pyrexia with neutrophil count under 1x10^9/l.

Question 22

How is neutropenic sepsis managed?

Answer 22

Immediate empirical broad spectrum IV antibiotics.

Tazocin, gentamicin.

Question 23

How is neutropenic sepsis prevented? (4).

Answer 23

Protective isolation.
Prophylactic antibiotics: levofloxacin.
Use of GCSF.
Hand hygiene.

Question 24

Who gets AML vs ALL?

Answer 24

ALL: 2-10 yr olds
AML: adults (risk increase with age)