الفهرس | Only 14 pages are availabe for public view |
Abstract the cause of chronic myeloid leukemia is a constitutively active BCR-ABL tyrosine kinase , It is a very rare disease in children, BMT is still the only curative therapy but TKI developed a new era in CML management. Subject and methods: this study was conducted on 98 children and adolescents diagnosed as CML during the period between January 1995 to December 2005 , in the Pediatric Hematology / Oncology Unit , Children Hospital , Ain Shams University , National Cancer Institute and Nasser Institute. We recorded the epidemiological features of the disease, symptoms, physical signs, peripheral blood and BM analysis at initial diagnosis, follow up patients till December 2008 was done, comparison between different TTT modalities was performed regarding OS,CHR,CR, duration till remission, duration till blast crisis, tolerance to therapy and outcome. Results: there was male preponderance with a ratio about 2:1 ,CML still extremely rare in very young children, >58% of patients were older than 10 years at diagnosis, an incidental diagnosis was made in 23.4% of patients with lower TLC and higher HB% than symptomatic group, about 6.1% of patients diagnosed in accelerated phase, 11.2% in blast crisis, the most common symptoms were athenia and symptoms related to splenomegaly ,imatinib and BMT were superior to other TTT modalities, imatinib was superior to BMT regarding outcome and toxicity profile. Conclusion: imatinib has to be regarded as standard of care first line TTT in pediatric CML but the long term outcome can not yet be assessed. On the other hand alloSCT holds the promise of cure but with definite toxicity and mortality. |