TRANSFORMATION OF MYELODYSPLASTIC SYNDROME TO T-CELL ACUTE LYMPHOBLASTIC LEUKEMIA IN A YOUNG ADULT

Chronic myeloid leukemia (CML) is infrequent in children. The best-known treatment is stem cell transplant. In a country with limited resources like Sudan, such expensive therapy is not available. Alternative approaches are needed to help these children. The tyrosine kinase inhibiter–imatinib–might be an answer to this problem. The objective of this study is to determine the pattern of children with CML, their hematological response to imatinib, and tolerance and side effects to this drug. All patients with confirmed BCR-ABL by polymerase chain reaction (PCR) were included in this study. The relevant data were collected and the patients were started on imatinib. Response to treatment was assessed clinically and hematologicaly only. Cytogentics and molecular studies are not available. The average age of the 31 patient evaluated was 8.7 years, 2 patients were less than 1 year, and 5 patients, ie, 16%, were 2 years old or less. Chloroma was observed in 6 (19%) patients. The average of the white blood cell (WBC) count was 206.6 × 10⁹/L and the platelet count average was 523 × 10⁹/L. Two (6.5%) of the 31 patients presented as acute myeloid leukemia (AML). All patients had hematological remission within 2 months. Twenty-three (74%) had a sustained remission over an average follow-up period of 26 months (2–67 months). Six (19%) patients died with AML or sepsis. Side effects to imatinib were infrequent, observed in 4 out of 29 (13.7%) patients, and mild. One patient only needed dose modification. No resistance was observed during this period. CML patients present at an earlier age than in other parts of the world. Imatinib is safe and effective in treating pediatric CML where stem cell transplant in not available. Further cytogentics are important to monitor response and proper management.