CML Flashcards
What is the average age of presentation in CML?
45-55 yo
What percentage of Adult Leukemias?
15%
What percentage is diagnosed via routine lab tests?
50%
What percentage is dignosed in the chronic phase?
85%
What are causes of CML?
- Idiopathic
- Radiation Exposure
- Chemical Exposure
What factors play a role in incidence of CML?
- Dose
2. Time of exposure
What are important lab findings in CML?
- Elevated Uric Acid
- Low LAP score (Benign granulocytes would have normal LAP score)
- Elevated B12 levels
- B12 Binding Protein elevation Transcobalamine 1
What is the terminal event in CML?
Blast crisis
What percentage transforms to AML?
2/3
What percentage transforms to ALL?
1/3
What is characteristic of CML?
Basophilia
What is the mutation in CML?
t(9;22) Philadelphia Chromosome
What does this translocation result in?
BCR-ABL fusion protein which results in increased tyrosine kinase activity.
What is the first line treatment for CML?
Imatinib TK inhibitor
What is the most prominent physical finding?
Splenomegaly sometimes due to metastasis
What percentage of CML patients show the t(9;22) translocation?
95%
Philadelphia Chromosome is present in
what percentage of RBC, WBC, Monocytes and platelets?
100%
What is the only known cure for CML?
Bone Marrow transplant.
What does imatinib effectively do?
Blocks the ATP binding site in the BCR-ABL fusion protein. Stopping the activity and generation of Tyrosine Kinase.
What is the bioavailability of Imatinib?
98%
What are the agents used in cases of Imatinab resistance?
Dasatinib & Nilotinib
What feature in PB smear is present only in CML?
Thrombocytosis.
