ALL in Infants

Infants account for approximately 5% of all children with ALL, and they experience the worst prognosis of any group of children with the disease. These patients have several poor prognostic features at diagnosis, including hyperleukocytosis, hepatomegaly, splenomegaly, and CNS leukemia.6 The bone marrow of infants at day 14 usually shows poor response to therapy. Infants with ALL have increased frequencies of cytogenetic abnormalities; 60% to 70% have a translocation that involves the MLL gene located at 11q23. The 11q23 breakpoint abnormality, t(4,11), is the most common structural karyotypic abnormality in infants with ALL. In vitro, blasts from infants with ALL showed greater drug resistance to prednisolone and L-asparaginase than those from older patients, although they are more sensitive to cytarabine.13 Based on this information, several studies are testing the efficacy of intensified chemotherapy that includes high-dose cytarabine. Another achievement is the prevention of CNS relapse using IT cytarabine in conjunction with high-dose systemic cytarabine. This combination has eliminated the need for cranial XRT in this young population. Even with major advances in cure rates for the general pediatric ALL population, where survival is 80% or more, the long-term EFS of infants is only about 40% (Tables 95-9 and 95-10).

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