Abstract
Down Sendromlu (DS; trizomi 21) infantlar, yenidoğan döneminde Transient Anormal Miyelopoez (TAM) olarak da bilinen, geçici myeloproliferatif hastalık gelişimi açısından risk altındadır. Transient Anormal Miyelopoez, DS klasik belirteçlerine sahip olan hastalarda görülebildiği gibi, Down Sendromu klasik belirteçlerine sahip olmayan mozaisizm gösteren hastalarda da ortaya çıkabilir. DS’lu yenidoğanların %4- 10’unda TAM gelişmektedir. TAM’lı bebeklerin çoğu remisyona girer ve herhangi bir tedavi gerekmez. Bu sunumda, kusma ve kanlı dışkılama şikayeti ile başvuran ve ileri değerlendirmeler sonucu; Mozaik Down Sendromu ve TAM tanıları konan yenidoğan olgusuna dikkat çekmek amaçlanmıştır.
Keywords
References
- 1-Massey GV. Transient leukemia in newborns with Down syndrome. Pediatr Blood Cancer 2005; 44:29–32.
- 2-Klusmann JH, Godinho FJ, Heitmann K, Maroz A, Koch ML, Reinhardt D, et al. Developmental stage specific interplay of GATA1 and IGF signaling in fetal megakaryopoiesis and leukemogenesis. Genes Dev 2010;24:1659-72.
- 3-Massey GV, Zipursky A, Chang MN, Doyle JJ, Nasim S, Taub JW, et al. A prospective study of the natural history of transient leukemia (TL) in neonates with Down syndrome (DS): Children’s Oncology Group (COG) study POG-9481. Blood 2006; 107: 4606–13.
- 4-Roberts I, Alford K, Hall G, Juban G, Richmond H, Norton A, et al. GATA1-mutant clones are frequent and often unsuspected in babies with Down syndrome: identification of a population at risk of leukemia. Blood 2013; 122: 3908–17.
Abstract
Infants with Down Syndrome (DS; trisomy 18) are under the risk for development of Transient Abnormal Myelopoiesis (TAM) during newborn period. TAM is mostly seen in classical form of DS but also might be seen in infants with mosaic type of DS. 4-10 % of infants with DS develop TAM. Most of infants with TAM, do not need any treatment and go into remission. Here, we tried to arouse interest on a newborn who has been consulted with compaints of vomiting and bloody stool and diagnosed as DownSyndrome with TAM.
Keywords
References
- 1-Massey GV. Transient leukemia in newborns with Down syndrome. Pediatr Blood Cancer 2005; 44:29–32.
- 2-Klusmann JH, Godinho FJ, Heitmann K, Maroz A, Koch ML, Reinhardt D, et al. Developmental stage specific interplay of GATA1 and IGF signaling in fetal megakaryopoiesis and leukemogenesis. Genes Dev 2010;24:1659-72.
- 3-Massey GV, Zipursky A, Chang MN, Doyle JJ, Nasim S, Taub JW, et al. A prospective study of the natural history of transient leukemia (TL) in neonates with Down syndrome (DS): Children’s Oncology Group (COG) study POG-9481. Blood 2006; 107: 4606–13.
- 4-Roberts I, Alford K, Hall G, Juban G, Richmond H, Norton A, et al. GATA1-mutant clones are frequent and often unsuspected in babies with Down syndrome: identification of a population at risk of leukemia. Blood 2013; 122: 3908–17.
Details
| Primary Language | Turkish |
|---|---|
| Subjects | Internal Diseases |
| Journal Section | CASE REPORTS |
| Authors | |
| Publication Date | December 23, 2019 |
| Submission Date | November 26, 2018 |
| Published in Issue | Year 2019 Volume: 13 Issue: 6 |
The publication language of Turkish Journal of Pediatric Disease is English.
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