Acute and Chronic Myelogenous Leukaemia (AML and CML)

Question 1

What does t(15:17) refer to?

Answer 1

The specific chromosomal translocation in acute promyelocytic leukaemia
(treated with ATRA)

Question 2

What other conditions is AML associated with?

Answer 2

Down’s
Radiation

Question 3

What is an important complication that can develop in patients with AML?

Answer 3

DIC
(Disseminated intravascular coagulation)

Question 4

What is DIC put simply?

Answer 4

Disseminated intravascular coagulation is widespread disruption in balance between coagulation and anticoagulation.
Small blood clots throughout bloodstream, consuming clotting factors and platelets. Therefore tendency to bleed elsewhere.
Typically secondary to another severe illness or trauma or complication during pregnancy.

Question 5

What is a typical leukaemia symptom?

Answer 5

Hepatosplenomegaly

Question 6

What are symptoms of AML?

Answer 6

General leukaemia symptoms (TATT, anaemia, infections, bleeding)

Gum infiltration
Hepatosplenomegaly

Question 7

How would you diagnose AML?

Answer 7

FBC and blood film:
Pancytopenia
Myeloperoxidase (MPO) positive
Auer rods

Bone marrow biopsy:
At least 20% myeloblasts

Question 8

What are Auer rods?

Answer 8

Myeloperoxidase (MPO) cytoplasmic aggregates in neutrophils

Question 9

What is treatment for AML?

Answer 9

Chemotherapy with allopurinol

ATRA (for the subtype acute promyelocytic leukaemia)

Consider antibiotics prophylaxis and transfusion

Last resort = bone marrow transplant

Question 10

Why is allopurinol given with chemotherapy in AML?

Answer 10

Prevents tumour lysis syndrome (TLS): Chemotherapy releases uric acid from the cells which can accumulate in the kidneys increasing risk of urate crystals forming, leading to kidney stones.

Question 11

What is the 3 year survival rate of AML?

Answer 11

20%
Very severe, rapid progression

Question 12

What age does AML mostly affect?

Answer 12

65 y/o

Question 13

What does t(9:22) refer to?

Answer 13

The specific chromosomal translocation found in CML (and subset of ALL).

BCR-ABL gene fusion encodes protein with abnormal tyrosine kinase activity (irreversibly switched on) leading to uncontrolled cell proliferation.

Question 14

CML is characterised by the presence of which chromosome, the result of the t(9:22) translocation.

Answer 14

Philadelphia chromosome

Question 15

What are symptoms of CML?

Answer 15

General leukaemia and hepatosplenomegaly

Question 16

What condition with CML would cause a massive hepatosplenomegaly?

Answer 16

malaria

Question 17

How would you diagnose CML?

Answer 17

FBC:
Pancytopenia (but granulocytosis)
See blood blast cell % to determine severity

BM biopsy:
increased granulocytes

Genetic testing:
Philadelphia chromosome

Question 18

According to WHO, what % cells of blood would need to be blasts to indicate CML as “chronic / phase 1”?

Answer 18

Up to 10%

Question 19

According to WHO, what is the range of blasts % of blood cells would indicate “accelerated/ phase 2” CML?

Answer 19

10-19%

Question 20

According to WHO< what % blood cells are blasts for it to be categorised as “blast crisis/ phase 3” in CML?

Answer 20

At least 20%

Question 21

What classification change would also happen during phase 3?

Answer 21

The leukaemia transforms into an acute leukaemia (AML)

Question 22

What is the treatment for CML?

Answer 22

Chemotherapy and imatinib
Consider allopurinol

Question 23

What is imatinib?

Answer 23

A tyrosine kinase inhibitor, decreases cell proliferation