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Clonal hematologic disorders in Down syndrome. A review

H Avet-Loiseau1, F Mechinaud, J L Harousseau

  • 1Department of Pediatric Hemato-Oncology, Hospital de la Mere et l'Enfant, Nantes, France.

Journal of Pediatric Hematology/Oncology
|February 1, 1995
PubMed
Summary

Children with Down syndrome (DS) have a higher risk of leukemia. Despite this, current treatments offer an excellent prognosis for these young patients, including for transient abnormal myelopoiesis (TAM).

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Area of Science:

  • Hematology
  • Pediatrics
  • Genetics

Background:

  • Children with Down syndrome (DS) exhibit an increased susceptibility to hematologic malignancies.
  • Understanding the specific risks and features of these disorders in DS is crucial for early detection and management.

Purpose of the Study:

  • To review and summarize data on leukemia incidence in children with Down syndrome.
  • To describe the phenotypic and functional characteristics of leukemic cells in this population.

Main Methods:

  • Evaluation of major leukemic subtypes: acute lymphoblastic leukemia (ALL), acute nonlymphoblastic leukemia (ANLL), myelodysplastic syndromes (MDS), and transient abnormal myelopoiesis (TAM).
  • Analysis in the context of patient age and treatment responsiveness.

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Main Results:

  • Leukemia is a relatively frequent event in children with DS.
  • Prognosis for leukemia in children with DS is excellent with current conventional therapies.

Conclusions:

  • Discusses treatment strategies for transient abnormal myelopoiesis (TAM), a neonatal monoclonal cellular proliferation.
  • Highlights the favorable outcomes achievable with existing therapeutic approaches for pediatric leukemia in Down syndrome.