Chronic Myeloid Leukaemia

Question 1

CML

Answer 1

Chronic myeloid leukaemia
Myeloproliferative neoplasm; characterised by a clonal proliferation of malignant myeloid progenitor cells resulting in an excessive number of myeloid cells in all stages of maturation
Arises in a primitive haemopoietic progenitor cell

Question 2

The Philadelphia chromosome

Answer 2

BCR chromosome 22, ABC chromosome 9 ]- Normal
BCR:ABL chromosome 22
Genetically evaluated by RT-PCR

Question 3

Philadelphia chromosome treatment

Answer 3

Hydroxyurea
Imatinib
Stem cell transplant
Dasatinib
- kinase inhibitor

Question 4

Imatinib in signal transduction inhibition

Answer 4

Targeted therapy
Blocks specific signalling pathways involved in growth and survival of cancer cells
Inhibits binding of ATP to ABL tyrosine kinase
Blocking downstream signalling pathways

Question 5

Dasatinib

Answer 5

Oral multi targeted kinase inhibitor
Multiple targets; BCR;ABL, Src family kinases ….
Superior to Imatinib

Question 6

Clinical features of CML

Answer 6

Hyper metabolism; splenomegaly; bleeding; gout; visual disturbances

Question 7

Lab findings in CML

Answer 7

Increased basophils and eosinophils; hyper cellular BM; Philadelphia chromosome; Leucocytosis; normocytic normochromic

Question 8

Genetic evaluation of BCR:ABL

Answer 8

RT-PCR
Light-cycler