Trisomy 21 associated transient neonatal myeloproliferation in the absence of Down's syndrome

Arch Dis Child Fetal Neonatal Ed. 1998 Nov;79(3):F215-7. doi: 10.1136/fn.79.3.f215.

Abstract

Although usually associated with Down's syndrome, transient neonatal myeloproliferation (TMD) can occur in the absence of a constitutional trisomy 21. This report describes two such cases, both of whom had a trisomy 21 restricted to clonal cells. Unlike in previous such reported cases, spontaneous morphological, cytogenetic, and molecular remission in both cases was followed by re-emergence, in one case, of an evolved clone with a more malignant phenotype which required pharmacological intervention. Awareness that trisomy 21 bearing leukaemia in the neonatal period can be transient even in the absence of Down's syndrome is important to prevent unnecessary treatment. Equally, such cases require indefinite follow up as a proportion may have a recurrence which may require treatment.

Publication types

  • Case Reports

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Chromosomes, Human, Pair 6
  • Cytarabine / administration & dosage
  • Down Syndrome*
  • Etoposide / administration & dosage
  • Female
  • Hematopoietic Stem Cells*
  • Humans
  • In Situ Hybridization, Fluorescence
  • Infant, Newborn
  • Karyotyping
  • Male
  • Mitoxantrone / administration & dosage
  • Myeloproliferative Disorders / drug therapy
  • Myeloproliferative Disorders / genetics*
  • Time Factors

Substances

  • Cytarabine
  • Etoposide
  • Mitoxantrone

Supplementary concepts

  • MAV protocol