Leukemia, AML & CML

Question 1

What is leukemia?

Answer 1

Neoplastic proliferation of WBC line (myeloblast/lymphoblast)

Question 2

what is a key point of leukemia?
What can the cells not do?

Answer 2

WBC/progenitors lose ability to differentiate (stuck as blast cells) but maintain ability to replicate

Question 3

Results of leukaemia

Answer 3

decreased production of other haematopoetic cells therefore function pancytopenia (increased infection risk, bleeding risk, anemia)

Question 4

Symptoms (generally) of leukaemia and why

Answer 4

BM failuren= Bone Pain
Bleeding (thrombocytopenia)
infections (leukopenia)
Anemia Sx (decreased RBC)
TATT (tired all the time)

Question 5

what does AML stand for?

Answer 5

Acute myelogenous leukaemia

Question 6

Definition of AML

Answer 6

neoplastic myeloblast (immature) proliferation - myeloid stem cell/myeloid blast

Question 7

What causes AML?

Answer 7

Translocation of chromosomes 15 & 17 t(15:17)

Question 8

What is AML associated with?

Answer 8

Down syndrome & radiation

Question 9

Most common subtype of AML?

Answer 9

Acute promyelocytic leukemia (APML)

Question 10

what happens if not treated?

Answer 10

rapid progression if not treated ASAP
3 year survival = only 20% therefore very severe

Question 11

Symptoms of AML?

Answer 11

General leukemia
Gum infiltration (swelling) - due to monocytes infiltration
Hepatosplenomegaly

Question 12

Diagnosis?
What tests are done and what results are seen?

Answer 12

Pancytopenia

Myeloblasts present which contain Auer rods (myeloperoxidase enzyme aggregates in neutrophils)

Myeloperoxidase positive (+ve)

BM Biopsy = >20% myeloid blasts

Question 13

What % of blasts are there usually in BM?

Answer 13

1-2%

Question 14

Treatment for AML

Answer 14

Chemo
ATRA (all trans retinoid acid) - used in subtype of AML; APML (gets rid of blasts -neutrophils from blood)

Consider Abx prophylaxis for neutrophilic, transfusion for anemia

Give allopurinol if doing chemo

Last resort - BM transplant

Question 15

Why would you give allopurinol if doing chemo?

Answer 15

prevents tumour lysis syndrome
(chemo releases uric acid from cells, can accumulate in kidneys)

Question 16

What is the difference between acute and chronic leukaemia?

Answer 16

Acute = don’t mature at all
chronic = mature partially

Question 17

definition of chronic myelogenous leukemia

Answer 17

Neoplastic myocyte proliferation - especially affect granulocytes (eosinophil, basophil, neutrophil)

Question 18

What causes CML

Answer 18

t(9:22)
Philadelphia chromosome - 22
On 22, BCR next to ABL, gene fusion BCR-ABL causing tyrosine kinase irreversibly switched ON

Question 19

What does tyrosine kinase do to cause leukemia?

Answer 19

Myeloid cells divide more quickly = high cell proliferation therefore build up
(divide too quickly)

Question 20

Symptoms of CML

Answer 20

general leukemia + massive hepatosplenomegaly

Question 21

Diagnosis of CML

Answer 21

FBC = pancytopenia (but granulocytosis)
BM Biopsy = increased granulocytes
*Philadelphia chromosome genetic test

Question 22

Blood blast cell % shows CML severity according to WHO:

Answer 22

<10 Chronic (1) Best
10-19 Accelerated (2)
>20 Blast crisis (3) Worst

Question 23

Treatment for CML

Answer 23

Chemo
Imantinib (T.K Inhibitor)
Consider allopurinol

Question 24

What is there a risk of in CML?

Answer 24

Progression to AML if untreated/late Dx = poorer prognosis
>20% blast cells on biopsy BM
Otherwise decent prognosis (if no progression)