Allogeneic Hematopoietic Cell Transplantation Ameliorated Asymptomatic Granulomatous and Lymphocytic Interstitial Lung Disease in a Patient With XIAP Deficiency.
J Pediatr Hematol Oncol
; 46(2): e191-e194, 2024 03 01.
Article
em En
| MEDLINE
| ID: mdl-38277621
ABSTRACT
X-linked inhibitor of apoptosis protein (XIAP) deficiency is an inborn error of immunity (IEI). Allogeneic hematopoietic cell transplantation (HCT) is currently the only curative therapy available for XIAP deficiency. Granulomatous and lymphocytic interstitial lung disease (GLILD) is a common immune-related lung complication of IEIs. We present a 6-year-old boy with XIAP deficiency and GLILD. Computed tomography showed lung nodes but no symptoms. Before HCT, GLILD was not managed with immunosuppressive therapy, because he was asymptomatic. The HCT procedure was subsequently performed. The post-HCT course was uneventful; follow-up computed tomography on day 46 showed nodules had disappeared. HCT could potentially ameliorate GLILD like other inflammatory processes associated with the underlying IEIs.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Imunodeficiência de Variável Comum
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Doenças Pulmonares Intersticiais
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Transplante de Células-Tronco Hematopoéticas
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Doenças Genéticas Ligadas ao Cromossomo X
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Transtornos Linfoproliferativos
Limite:
Child
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Humans
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Male
Idioma:
En
Revista:
J Pediatr Hematol Oncol
Ano de publicação:
2024
Tipo de documento:
Article