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Pediatric chronic myeloid leukemia constitutes around 3-5% of all childhood malignancies. It is characterized by t(9;22) with BCR-ABL1 fusion and p210 transcript. A 9-year-old male child presented with hepatosplenomegaly, anemia, marked leucocytosis, basophilia, myelocyte metamyelocyte peak, and 4% blasts in the differential count. Leukocyte alkaline phosphatase score was reduced and p210 transcript of BCR-ABL1 was identified by a polymerase chain reaction. Bone marrow was hypercellular with increased granulopoiesis, dyspoietic megakaryocytes and grade III reticulin fibrosis. The patient was treated with imatinib and showed a hematological response within one month and has a stable disease for the last 24 months. Owing to lack of specific guidelines on management and monitoring of pediatric chronic myeloid leukemia, it managed according to the adult guidelines by European leukemia Net or National Comprehensive Cancer Network guidelines. Drug toxicities, effect on growth, vaccination, and fertility are pressing issues in management of pediatric chronic myeloid leukemia with current first-line therapy with tyrosine kinase inhibitors.