Chronic Myeloid Leukaemia with the newest treatment

Chronic Myeloid Leukaemia

•          CML is a clonal disorder of pluripotent stem cells

•          One of MPD that forms 15-20% of all leukaemias.

•          Peak age of incidence 40-60ys

•          Incidence : 1.25 for 100,000

•          The majority die within 5 years

•          3 phases :

     1- Chronic phase

     2- Accelerated phase

     3- Blastic phase

•          Clinical picture:

•          Asymptomatic discovered accidentally

•          Anaemia

•          Abdominal discomfort due to splenic enlargement (Splenomegaly in > 75%), hepatomegaly

•          Sweating, fever, wt loss

•          Signs:   Pallor and Splenomegaly that can be huge•     DD: * Reactive leucocytosis ( leukomoid reaction), * Other MPD as PRV and in our country  *Bilharzial splenomegaly

Investigations

•          Peripheral blood :

•          Hb N or ¯

•          Plt : N , ­ or ¯

•          WCC is ­ ( usually above 50,000/cu mm but may reach up to 500,000) shows the whole spectrum of the myeloid precursors with only few blasts

•          BM: hypercellular with ­  in myeloid series

•          Cytogenetics Ph chromosome is present in most of the pts

•          Management of CML
Imatinib ;STI 571 ( Tyrosine kinase inhibitor)is the first line drug in the management of chronic phase ( causes major cytogenic response in 83% of patients)

•          Chemotherapy : Hydroxyurea , it is effective in controlling the leucocytic count

•          IFN-alpha SC may produce cytogenetic response ( decrease the % of Ph +ve cells)

•          All. BMT: is the only established curative treatment