Chronic Leukaemia (additional bits not in leukaemia)

Question 1

What is a philadelphia chromosome?

Answer 1

Reciprocal Translocation of long arms of 9 and 22 t(9:22)

Question 2

How does the philadelphia chromosome lead to CML?

Answer 2

BCR-ABL fusion gene made –> strong tyrosine kinase activity encoded –> myeloid cells divide quicker

Question 3

What are the 3 stages of CML?

Answer 3

Chronic Phase

Accelerated Phase

Blast Crisis

Question 4

When are most CML’s diagnosed?

Answer 4

in the chronic phase - incidentally found on routine bloods

Question 5

When is CML determined to be in the accelerated phase?

Answer 5

15-29% blasts
>20% basophils
thrombocytopaenia

more systemic symptoms

Question 6

What is a blast crisis?

Answer 6

No ability to differentiate so resemble acute leukaemia

Bone marrow exhaustion and large tumour burden - systemically unwell and rapidly fatal

Question 7

How does CML progress?

Answer 7

Years between chronic and accelerated phase

Months between accelerated and blast crisis

Question 8

What are the goals of management in CML?

Answer 8

Haematological remission - FBC and symptoms improve

Cytogenetic Remission - 0% Ph+vs cells

Molecular remission - PCR-ve for BCR-ABL

Question 9

What is regularly monitored throughout management of CML?

Answer 9

FISH studies - show % bone marrow cells Ph+