CML t(9;22) Chronic Myelogenous Leukemia

Question 1

What is a t(9:22) also called?

Answer 1

Philadelphia Chromosome

Question 2

WHat is the fusion product of the philadelphia chromosome?

Answer 2

BCR-ABL1….uninhibited Tyrosine Kinase Activity

Question 3

What are the 4 phases?

Answer 3

Acute, Chronic, accelerated, blast phase. The earlier you catch it the better

Question 4

What is the treatment>

Answer 4

Imatinib.

Question 5

How do you diagnose CML

Answer 5

RT-PCR assay for the BCR-ABL1 gene fusion.

Question 6

Why should you also get a BM biopsy for CML patients?

Answer 6

B/C if the CML has progressed to the blast phase it can turn into Acute Myelogenous Leukemia which needs to be treated differently.

Question 7

Who is mostly affected?

Answer 7

30-60 year olds.

Question 8

Morphology>

Answer 8

BM= hypercellular

Unexplained basophilia in peripheral blood

Question 9

Name the 5 diseases which fit into the chronic myeloproliferative disease category.

Answer 9

Chronic myelogenous leukemia, Essential thrombocythemia, Polycythemia vera, Mastocytosis, Primary myelofibrosis.

Question 10

Side effects of Imatinib

Answer 10

nausea, edema, skin rash, muscle cramps

Question 11

How does Imatinib work?

Answer 11

Inhibits the Tyrosine kinase activity of BCR-ABL by blocking the ATP binding site.