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Case Reports
. 2022 Apr;120(2):e89-e92.
doi: 10.5546/aap.2022.e89. Epub 2022 Feb 4.

[Myeloproliferative disorders and leukemia related to Down syndrome. Report of two clinical cases in neonatal period]

[Article in Spanish]
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Free article
Case Reports

[Myeloproliferative disorders and leukemia related to Down syndrome. Report of two clinical cases in neonatal period]

[Article in Spanish]
María X Castro et al. Arch Argent Pediatr. 2022 Apr.
Free article

Abstract

Down syndrome predisposes to haematological disorders. It is estimated that 5-30% of neonates with this condition will develop transient abnormal myelopoiesis. Treatment is not standardized; exchange transfusion and the use of cytarabine could be effective. We present two clinical cases of patients with Down syndrome, who during the neonatal period showed acute myeloid leukemia and transient abnormal myelopoiesis, the treatments used and their outcomes. Suspicion and early diagnosis of this entity are considered determining factors in prognosis.

El síndrome de Down predispone a trastornos mieloproliferativos. Se estima que del 5 % al 30 % de los neonatos con esta condición desarrollarán mielopoyesis anormal transitoria. El tratamiento no está estandarizado; la exanguinotransfusión y la citarabina podrían ser efectivos. Se describen dos casos de pacientes con síndrome de Down, quienes durante el período neonatal presentaron leucemia mieloide aguda y mielopoyesis anormal transitoria, los tratamientos utilizados y sus desenlaces. Se considera que la sospecha y el diagnóstico temprano de esta entidad son factores determinantes en el pronóstico.

Keywords: Down syndrome; leukemia; transient myeloproliferative syndrome; tumor lysis syndrome.

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References

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    1. Tandonnet J, Clavel J, Baruchel A, Nacka F, Pérel Y. Myeloid leukaemia in children with Down syndrome: report of the registry-based French experience between 1990 and 2003. Pediatr Blood Cancer. 2010; 54(7):927-33.
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    1. Okamura T, Washio Y, Yoshimoto J, Tani K, et al. Exchange Transfusion and Cytarabine for Transient Abnormal Myelopoiesis in Hydrops Fetalis. Acta Med Okayama. 2019; 73(2):181-8.
    1. Watanabe K. Recent advances in the understanding of transient abnormal myelopoiesis in Down syndrome. Pediatr Int. 2019; 61(3):222-9.

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