Tratamiento de anemia aplásica severa adquirida en pacientes pediátricos con inmunosupresión y trasplante alogénico de precursores hematopoiéticos

Background: Severe acquired aplastic anemia (SAA) is an uncommon disease of childhood. Patients with SAA receive supportive care with transfusions and timely treatment of opportunistic infections, along with specific therapies, which may be allogenic stem cell transplantation (SCT) from a matched si...

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Autores principales:	Barriga C,Francisco, Wietstruck P,Angélica, Becker K,Anna, Zúñiga C,Pamela, Besa De C,Pelayo, Alvarez Z,Manuel, Ocqueteau T,Mauricio, Bertín C,Pablo
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2007
Materias:	Anemia, aplastic Immunosupression Stem cell transplantation
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872007001100008
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spelling	oai:scielo:S0034-988720070011000082008-02-08Tratamiento de anemia aplásica severa adquirida en pacientes pediátricos con inmunosupresión y trasplante alogénico de precursores hematopoiéticosBarriga C,FranciscoWietstruck P,AngélicaBecker K,AnnaZúñiga C,PamelaBesa De C,PelayoAlvarez Z,ManuelOcqueteau T,MauricioBertín C,Pablo Anemia, aplastic Immunosupression Stem cell transplantation Background: Severe acquired aplastic anemia (SAA) is an uncommon disease of childhood. Patients with SAA receive supportive care with transfusions and timely treatment of opportunistic infections, along with specific therapies, which may be allogenic stem cell transplantation (SCT) from a matched sibling or immunosupressive therapy (IT). Aim: To report the experience in the management of SAA. Patients and methods: Twenty five children with acquired SAA were treated from July 1992 to September 2005. Patients with full matched sibling donors received allogenic SCT after conditioning with a cyclophosphamide containing regimen. The other patients received immune suppression with cyclosporine plus methylprednisolone (n= 18) plus ATG (n=17). All received supportive care until recovery of hematopoietic function. Those who had severe opportunistic infections at diagnosis or did not respond to two cycles of ATG were evaluated for unrelated donor SCT. Results: Seven patients received sibling donor SCT and 18 IT, which was repeated in six. Three patients received mismatched related (1) or unrelated (2) SCT. Nineteen patients survived with a median follow up time of 4 years, 14 with full hematologic recovery. Six patients died: four due to infections after IT or SCT, one due to intracranial hemorrhage and one with secondary myelodysplasia 12 years after IT. Conclusions: Most children with SAA can be treated successfully with sibling donor SCT or IT. Patients without a histocompatible sibling who fail to respond to IS have a worse prognosisinfo:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.135 n.11 20072007-11-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872007001100008es10.4067/S0034-98872007001100008
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Anemia, aplastic Immunosupression Stem cell transplantation
spellingShingle	Anemia, aplastic Immunosupression Stem cell transplantation Barriga C,Francisco Wietstruck P,Angélica Becker K,Anna Zúñiga C,Pamela Besa De C,Pelayo Alvarez Z,Manuel Ocqueteau T,Mauricio Bertín C,Pablo Tratamiento de anemia aplásica severa adquirida en pacientes pediátricos con inmunosupresión y trasplante alogénico de precursores hematopoiéticos
description	Background: Severe acquired aplastic anemia (SAA) is an uncommon disease of childhood. Patients with SAA receive supportive care with transfusions and timely treatment of opportunistic infections, along with specific therapies, which may be allogenic stem cell transplantation (SCT) from a matched sibling or immunosupressive therapy (IT). Aim: To report the experience in the management of SAA. Patients and methods: Twenty five children with acquired SAA were treated from July 1992 to September 2005. Patients with full matched sibling donors received allogenic SCT after conditioning with a cyclophosphamide containing regimen. The other patients received immune suppression with cyclosporine plus methylprednisolone (n= 18) plus ATG (n=17). All received supportive care until recovery of hematopoietic function. Those who had severe opportunistic infections at diagnosis or did not respond to two cycles of ATG were evaluated for unrelated donor SCT. Results: Seven patients received sibling donor SCT and 18 IT, which was repeated in six. Three patients received mismatched related (1) or unrelated (2) SCT. Nineteen patients survived with a median follow up time of 4 years, 14 with full hematologic recovery. Six patients died: four due to infections after IT or SCT, one due to intracranial hemorrhage and one with secondary myelodysplasia 12 years after IT. Conclusions: Most children with SAA can be treated successfully with sibling donor SCT or IT. Patients without a histocompatible sibling who fail to respond to IS have a worse prognosis
author	Barriga C,Francisco Wietstruck P,Angélica Becker K,Anna Zúñiga C,Pamela Besa De C,Pelayo Alvarez Z,Manuel Ocqueteau T,Mauricio Bertín C,Pablo
author_facet	Barriga C,Francisco Wietstruck P,Angélica Becker K,Anna Zúñiga C,Pamela Besa De C,Pelayo Alvarez Z,Manuel Ocqueteau T,Mauricio Bertín C,Pablo
author_sort	Barriga C,Francisco
title	Tratamiento de anemia aplásica severa adquirida en pacientes pediátricos con inmunosupresión y trasplante alogénico de precursores hematopoiéticos
title_short	Tratamiento de anemia aplásica severa adquirida en pacientes pediátricos con inmunosupresión y trasplante alogénico de precursores hematopoiéticos
title_full	Tratamiento de anemia aplásica severa adquirida en pacientes pediátricos con inmunosupresión y trasplante alogénico de precursores hematopoiéticos
title_fullStr	Tratamiento de anemia aplásica severa adquirida en pacientes pediátricos con inmunosupresión y trasplante alogénico de precursores hematopoiéticos
title_full_unstemmed	Tratamiento de anemia aplásica severa adquirida en pacientes pediátricos con inmunosupresión y trasplante alogénico de precursores hematopoiéticos
title_sort	tratamiento de anemia aplásica severa adquirida en pacientes pediátricos con inmunosupresión y trasplante alogénico de precursores hematopoiéticos
publisher	Sociedad Médica de Santiago
publishDate	2007
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872007001100008
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Tratamiento de anemia aplásica severa adquirida en pacientes pediátricos con inmunosupresión y trasplante alogénico de precursores hematopoiéticos

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