It tends to affect middle aged people (40 to 60 years)
Classical presentation is with:
- decreased weight
Possible additional presentations are:
- gout due to increased purine breakdown
- abdo pain due to splenomegaly
- bleeding due to platelet dysfunction
Approximately 30% are detected by chance.
- elevated WCC - often 100-500
- massive neutrophilia with left shift
- low neutrophil alkaline phosphatase
- splenomegaly in >75%
The philadelphia chromosome is present in >80% of cases
The philadelphia chromosome:
- is a hybrid chromosome formed by t(9;22)
- forms a gene BCR-ABL which results in excess tyrosine kinase activity
- found in 80% CML, 5% children with ALL, 25% of adult ALL and 1% adult AML.
- is associated with a good prognosis in CML but a poor prognosis in all other leukaemias
Treatment for CML is:
- imatimib = glivec = specific BCR-ABL tyrosine kinase inhibitor
- bone marrow transplant
CML transforms to AML in 80% of cases and ALL in 20%
Last battle of the day is Gertmann's Syndrome...