Clinical and biological aspects of myeloid leukemia in Down syndrome.

Autor: Boucher AC; Department of Hematology, St. Jude Children's Research Hospital, Memphis, TN, 38105, USA., Caldwell KJ; Department of Oncology, St. Jude Children's Research Hospital, Memphis, TN, 38105, USA., Crispino JD; Department of Hematology, St. Jude Children's Research Hospital, Memphis, TN, 38105, USA. John.crispino@stjude.org., Flerlage JE; Department of Oncology, St. Jude Children's Research Hospital, Memphis, TN, 38105, USA. Jamie.flerlage@stjude.org.
Jazyk: angličtina
Zdroj: Leukemia [Leukemia] 2021 Dec; Vol. 35 (12), pp. 3352-3360. Date of Electronic Publication: 2021 Sep 13.
DOI: 10.1038/s41375-021-01414-y
Abstrakt: Children with Down syndrome are at an elevated risk of leukemia, especially myeloid leukemia (ML-DS). This malignancy is frequently preceded by transient abnormal myelopoiesis (TAM), which is self-limited expansion of fetal liver-derived megakaryocyte progenitors. An array of international studies has led to consensus in treating ML-DS with reduced-intensity chemotherapy, leading to excellent outcomes. In addition, studies performed in the past 20 years have revealed many of the genetic and epigenetic features of the tumors, including GATA1 mutations that are arguably associated with all cases of both TAM and ML-DS. Despite these advances in understanding the clinical and biological aspects of ML-DS, little is known about the mechanisms of relapse. Upon relapse, patients face a poor outcome, and there is no consensus on treatment. Future studies need to be focused on this challenging aspect of leukemia in children with DS.
(© 2021. The Author(s), under exclusive licence to Springer Nature Limited.)
Databáze: MEDLINE
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