[Haem] Leukaemia Flashcards
what is chronic myeloid leukaemia (CML) associated with?
- dysregulated production of granulocytes
- ‘Philadelphia chromosome’ t(9;22) a balanced reciprocal translocation, in 90-95%
how does CML present?
- > 65 years
- chronic phase then transforms to an accelerated phase
- non-specific sx: fatigue, MASSIVE splenomegaly
what can be seen biochemically for CML?
- normocytic anaemia
- neutrophilia
- absolute basophilia (>0.1x10^9/L)
how is a dx of CML made?
- blood film
- bone marrow biopsy
- cytogenetic analysis
what does a bone marrow biopsy for CML show?
granulocytic hyperplasia with cells of all maturity
what is cytogenetic analysis done for in CML?
FISH done to confirm presence of BCR-ABL1 fusion gene
what is the 1st line rx for CML?
Imatinib, a 1st gen tyrosine kinase inhibitor
a CML dx is made and pt is in a chronic phase. how is this treated?
Imatinib
a pt is in a chronic phase of CML and was treated with Imatinib but showed no response. what is given next?
2nd gen tyrosine kinase inhibitor e.g. dasatinib
a CML dx is made and pt is in an accelerated phase. how is this treated?
Imatinib and search for stem cell donor
a pt is in an accelerated phase of CML and was treated with Imatinib but showed no response. how is this pt treated next?
stem cell transplant
what is the accelerated or blastic phase of CML defined by?
- high blast count 10-19%
- basophil count >20%
- progressive splenomegaly / WCC unresponsive to tyrosine kinase therapy
from where does the Philadelphia chromosome result from?
from the fusion of BCR-ABL1 t(9;22)
what does the fusion of BCR-ABL1 gene result in?
increased tyrosine kinase activity
what is the significance of the Philadelphia chromosome if present in acute lymphocytic leukaemia (ALL)?
poor Px
