Increased complications and morbidity in children with hemophagocytic lymphohistiocytosis undergoing hematopoietic stem cell transplantation.
Clin Transplant
; 27(2): 248-54, 2013.
Article
em En
| MEDLINE
| ID: mdl-23331022
ABSTRACT
BACKGROUND:
Hematopoietic stem cell transplantation (HSCT) is the only curative option for patients with primary hemophagocytic lymphohistiocytosis (HLH) and for patients with secondary HLH who fail to respond to therapy.METHODS:
Retrospective study of HSCT for HLH with focus on complications and outcome.RESULTS:
Eighteen children (10 males), with a median age of 1.2 yr (5 months-16 yr), received HSCT for HLH. Fourteen children had primary HLH. Four children underwent transplant while not in remission. Sixteen received myeloablative and two received reduced intensity conditioning regimen. A high incidence of complications was found 13 (72%) children had 22 episodes of culture-proven infections; seven (38%) had hepatic veno-occlusive disease; nine (50%) developed respiratory complications; and nine (50%) required intensive care unit admission. Eight children had acute graft-versus-host disease (GVHD), and three developed chronic GVHD. Three patients died from multi-organ failure before day +100, and another patient died from pulmonary hemorrhage after day 100. Three patients failed to engraft (two developed recurrent HLH and died from complications after a second HSCT). Three of four children not in remission at the time of transplantation died. Actuarial survival at three yr was 61%.CONCLUSION:
HSCT for HLH carries significant risks with high infection, organ dysfunction, and ICU admissions rates.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Complicações Pós-Operatórias
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Transplante de Células-Tronco Hematopoéticas
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Linfo-Histiocitose Hemofagocítica
Tipo de estudo:
Evaluation_studies
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Incidence_studies
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Observational_studies
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Prognostic_studies
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Risk_factors_studies
Limite:
Adolescent
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Child
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Child, preschool
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Female
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Humans
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Infant
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Male
Idioma:
En
Revista:
Clin Transplant
Assunto da revista:
TRANSPLANTE
Ano de publicação:
2013
Tipo de documento:
Article
País de afiliação:
Canadá