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Heamatology summary 14


Chapter 14 in Haematology (chronic myeloid leukaemia) summary         

 

-Chronic myeloid leukaemia is a clonal disorder of a pluripotent steam cell. The disease accounts for around 15 %of leukamias and may occur at anu age.

-All cases of CML have a transloction between chromosomes 9 and 22. This leads to the BCP hene on chrosome 22 and generates the philadelphia chromosome.

-The resulting chimeric BCR-ABL1 gene codes for a fusion protien with tyrosine kinase activity.

-In some patients the phildaelphia examination of tumour cells but the only be detected by FISH or PCR.

-The disease can occur at any age but is most common between the ages of 40 and 60 years.

-The clinical feauters include anaemia bleeding and splenomegaly. There is usually a marked neutrophlia with myelocytes and basophils seen in the blood film.

-Transformation to an accelerated phase or acyte leukaemia may occur.

-Treatement is with tyrosine kinase inhibitors such as imatinib,dasatinib or nilotinib.tumour cells can acquired resistance totreatment and drug therapy is tailpred in response to this.

-Stem cell transplantation can be curative and may also be useful for advanced disease.

-The clinical outlook is now very good and patient can expect long-term control of disease.

-Chronic eosinophilic and neutrophil leukaemia are much rare.

 

From book: Essentail Haematology A.V.HOFFBRAND,P.A.H.MOSS.6EDITION
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