Tratamiento de anemia aplásica severa adquirida en pacientes pediátricos con inmunosupresión y trasplante alogénico de precursores hematopoiéticos / Treatment of acquired severe aplastic anemia in pediatric patients with immunosupression and allogeneic stem cell hematopoyetic transplant
Rev. méd. Chile
;
135(11): 1421-1428, nov. 2007. ilus, tab, graf
Artigo
em Espanhol
| LILACS
| ID: lil-472841
ABSTRACT
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 andmethods:
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.
Texto completo:
DisponíveL
Índice:
LILACS (Américas)
Assunto principal:
Transplante de Células-Tronco Hematopoéticas
/
Imunossupressores
/
Anemia Aplástica
Tipo de estudo:
Ensaio Clínico Controlado
/
Estudo de etiologia
/
Estudo observacional
/
Estudo prognóstico
/
Fatores de risco
Limite:
Adolescente
/
Criança
/
Criança, pré-escolar
/
Feminino
/
Humanos
/
Masculino
Idioma:
Espanhol
Revista:
Rev. méd. Chile
Assunto da revista:
Medicina
Ano de publicação:
2007
Tipo de documento:
Artigo
País de afiliação:
Chile
Instituição/País de afiliação:
Hospital Sótero del Rio/CL
/
Pontificia Universidad Católica de Chile/CL
Similares
MEDLINE
...
LILACS
LIS