Transient Myeloproliferative Disorder in Neonates with Down syndrome: case report and review

Down syndrome (DS) or trisomy 21 is the most prevalent chromosomopathy. Interesting associations have been documented between DS and various hematopoietic and non-hematopoietic malignancies. Transient myeloproliferative disorder (TMD) is a clonal proliferation of megakaryoblasts, typically occurrin...

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Autores principales: Lerda, Daniel Enrique, Polacov, Silvana Micaela, Bertoldi, Ariel Enrique, Hollmann, Carlos Hernan
Formato: Artículo acceptedVersion
Lenguaje:Inglés
Publicado: Longdom Group SA 2018
Materias:
R Medicina (General)
RJ Pediatría
RJ101 Salud del Niño. Servicios de salud infantil
Acceso en línea:http://pa.bibdigital.ucc.edu.ar/1463/1/A_Polacov_Bertoldi_Lerda.pdf
Aporte de:
Producción Académica Universidad Católica de Córdoba (UCCor) de Universidad Católica de Córdoba
id I38-R144-1463
record_format dspace
institution Universidad Católica de Córdoba
institution_str I-38
repository_str R-144
collection Producción Académica Universidad Católica de Córdoba (UCCor)
language Inglés
orig_language_str_mv eng
topic R Medicina (General)
RJ Pediatría
RJ101 Salud del Niño. Servicios de salud infantil
spellingShingle R Medicina (General)
RJ Pediatría
RJ101 Salud del Niño. Servicios de salud infantil
Lerda, Daniel Enrique
Polacov, Silvana Micaela
Bertoldi, Ariel Enrique
Hollmann, Carlos Hernan
Transient Myeloproliferative Disorder in Neonates with Down syndrome: case report and review
topic_facet R Medicina (General)
RJ Pediatría
RJ101 Salud del Niño. Servicios de salud infantil
description Down syndrome (DS) or trisomy 21 is the most prevalent chromosomopathy. Interesting associations have been documented between DS and various hematopoietic and non-hematopoietic malignancies. Transient myeloproliferative disorder (TMD) is a clonal proliferation of megakaryoblasts, typically occurring in newborns with DS. It is believed that TMD occurs in the presence of GATA-1 mutation together with trisomy 21. The disorder resolved in the majority of patients during the first six months of life, however, 30% of patients can develop acute leukemia or a myelodysplastic syndrome in the first five years of life. In most instances, this unique disorder has the ability to spontaneously “turn off” the overproliferation and enter a state of remission. Only supportive care is recommended for TMD during the first months of life unless the clinical condition requires intervention. This is the report of a preterm newborn with Down syndrome diagnosed with TMD that required chemotherapy on the first days of life due to a poor clinical course and other risk factors for early death. As more cases of this disorder are presented, it is important to share our experience to aid in management and diagnosis.
format Artículo
Artículo
acceptedVersion
author Lerda, Daniel Enrique
Polacov, Silvana Micaela
Bertoldi, Ariel Enrique
Hollmann, Carlos Hernan
author_facet Lerda, Daniel Enrique
Polacov, Silvana Micaela
Bertoldi, Ariel Enrique
Hollmann, Carlos Hernan
author_sort Lerda, Daniel Enrique
title Transient Myeloproliferative Disorder in Neonates with Down syndrome: case report and review
title_short Transient Myeloproliferative Disorder in Neonates with Down syndrome: case report and review
title_full Transient Myeloproliferative Disorder in Neonates with Down syndrome: case report and review
title_fullStr Transient Myeloproliferative Disorder in Neonates with Down syndrome: case report and review
title_full_unstemmed Transient Myeloproliferative Disorder in Neonates with Down syndrome: case report and review
title_sort transient myeloproliferative disorder in neonates with down syndrome: case report and review
publisher Longdom Group SA
publishDate 2018
url http://pa.bibdigital.ucc.edu.ar/1463/1/A_Polacov_Bertoldi_Lerda.pdf
work_keys_str_mv AT lerdadanielenrique transientmyeloproliferativedisorderinneonateswithdownsyndromecasereportandreview
AT polacovsilvanamicaela transientmyeloproliferativedisorderinneonateswithdownsyndromecasereportandreview
AT bertoldiarielenrique transientmyeloproliferativedisorderinneonateswithdownsyndromecasereportandreview
AT hollmanncarloshernan transientmyeloproliferativedisorderinneonateswithdownsyndromecasereportandreview
bdutipo_str Repositorios
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