Leukemia, chronic myeloid

They account for 2 to 3 % of the leukemias in childhood. In 99 % of cases, there is a specific translocation [t(9;22)(q34;q11)] of the BCR-ABL gene of tyrosine-kinase, called the Philadelphia chromosome. The malignant cell clone produces a high rate of leukocytes with a predominance of mature forms but a great amount of immature granulocytes. Clinically, the disease begins insidiously. Progressive splenomegaly develops and then becomes painful when the upper quadrant of the abdomen is palpated. As hyperleucocytosis increases, anemia and thrombopenia develop.


Typically, the insidious chronic phase lasts 3-4 years before a brutal acutization called a "blast crisis". Hyperleucocytosis becomes very severe and is no longer controllable by agents such as hydroxyurea. Significant hyperuricemia and neurological disorders related to increased blood viscosity appear. General discomfort, fever, asthenia, anorexia, weight loss complete the picture.

Diagnosis is made on blood analysis and bone marrow cytology, and molecular biology, and the presence of the Philadelphia chromosome (which can sometimes be found in some forms of ALL and acute myelocytic leukemia). Specific anti-tyrosine-kinases (imatinib, dasatinib, nilotinib, etc.) are effective in the long term in more than 70 % of cases but have significant side effects (including cardiotoxicity).


Anesthetic implications

see acute lymphoblastic leukemias


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Updated: March 2021