Abstract • A serial clinical, hematologic, and cytogenetic study was done on a baby with Down's syndrome in whom a myeloid leukemoid reaction developed at birth that spontaneously regressed within a month only to relapse two years later to an acute undifferentiated stem cell leukemia. He died 1½ months after onset. The unresolved controversy of the diagnosis of the congenital leukemia-like state is discussed. The importance of following up such patients with apparent remission of their congenital leukemia-like disorder is emphasized. (Am J Dis Child 134:939-941, 1980) References 1. Krivit W, Good RA: Simultaneous occurrence of mongolism and leukemia . Am J Dis Child 94:289-293, 1957. 2. Wald N, Borges WL, Li CC, et al: Leukaemia associated with mongolism . Lancet 1:1228, 1961.Crossref 3. Ross JD, Maloney WC, Desforges JF: Ineffective regulation of granulopoiesis masquerading as congenital leukemia in a mongoloid child . J Pediatr 63:1-10, 1963.Crossref 4. Engel RR, Hammond D, Eitzman DV, et al: Transient congenital leukemia in seven infants with mongolism . J Pediatr 65:303-305, 1964.Crossref 5. Honda F, Punett HH, Charney E, et al: Serial cytogenetic and hematologic studies on a mongol with trisomy 21 with acute congenital leukemia . J Pediatr 65:880-887, 1964.Crossref 6. Conen PE, Erkman B: Combined mongolism and leukemia: Report of eight cases with chromosome studies . Am J Dis Child 112:429-443, 1966. 7. Kauffman HJ, Hess R: Does congenital leukaemia exist? Br Med J 1:867-868, 1966.Crossref 8. Rosner F, Lee SL, written for the Acute Leukemia Group B: Down's syndrome and acute leukemia: Myeloblastic or lmyphoblastic? Report of 43 cases and review of the literature . Am J Med 53:203-218, 1972.Crossref 9. Schunk GJ, Lehman LW: Mongolism and congenital leukemia . JAMA 155:250-251, 1954.Crossref 10. O'Connor RE, McKay RN, Smith J: Congenital leukemia with septicemia as a terminal event . Am J Dis Child 88:740-742, 1954. 11. Weinberger MM, Oleinick A: Congenital marrow dysfunction in Down's syndrome . J Pediatr 77:273-279, 1970.Crossref 12. Miller JM, Sherrill JG, Hathaway WE: Thrombocythemia in the myeloproliferative disorder of Down's syndrome . Pediatrics 40:847-850, 1967. 13. Nagao T, Lampkin BC, Hug G: A neonate with Down's syndrome and transient abnormal myelopoiesis: Serial blood and bone marrow studies . Blood 36:443-447, 1970. 14. Rosner F, Ong BH, Paine RS, et al: Biochemical differentiation of trisomic Down's syndrome (mongolism) from that due to translocation . N Engl J Med 273:1356-1361, 1965.Crossref
American Journal of Diseases of Children – American Medical Association
Published: Oct 1, 1980
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