Successful living donor liver transplantation for liver failure due to maternal T cell engraftment following cord blood transplantation in X-linked severe combined immunodeficiency disease: Case report.
Am J Transplant
; 21(9): 3184-3189, 2021 09.
Article
em En
| MEDLINE
| ID: mdl-33793086
ABSTRACT
Maternal T cells from perinatal transplacental passage have been identified in up to 40% of patients with severe combined immunodeficiency (SCID). Although engrafted maternal T cells sometimes injure newborn tissue, liver failure due to maternal T cells has not been reported. We rescued a boy with X-linked SCID who developed liver failure due to engrafted maternal T cell invasion following living donor liver transplantation (LDLT) following unrelated umbilical cord blood transplantation (UCBT). After developing respiratory failure 3 weeks postpartum, he was diagnosed with X-linked SCID. Pathological findings showed maternal T cells engrafted in his liver and hepatic fibrosis gradually progressed. He underwent UCBT at 6 months, but hepatic function did not recover and liver failure progressed. Therefore, he underwent LDLT using an S2 monosegment graft at age 1.3 years. The patient had a leak at the Roux-en-Y anastomosis, which was repaired. Despite occasional episodes of pneumonia and otitis media, he is generally doing well 6 years after LDLT with continued immunosuppression agents. In conclusion, the combination of hematopoietic stem cell transplantation (HSCT) and liver transplantation may be efficacious, and HSCT should precede liver transplantation for children with X-linked SCID and liver failure.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Transplante de Fígado
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Falência Hepática
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Transplante de Células-Tronco Hematopoéticas
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Transplante de Células-Tronco de Sangue do Cordão Umbilical
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Doenças por Imunodeficiência Combinada Ligada ao Cromossomo X
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Doença Enxerto-Hospedeiro
Tipo de estudo:
Prognostic_studies
Limite:
Female
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Humans
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Infant
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Male
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Pregnancy
Idioma:
En
Ano de publicação:
2021
Tipo de documento:
Article