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Article

Autosomal dominant hyper-IgE syndrome: When hematopoietic stem cell transplantation should be considered?

Pediatric transplantation - 1 Aug 2020

Oikonomopoulou Christina, Goussetis Evgenios

Abstract excerpt

AD-HIES or Job's syndrome is a primary immunodeficiency, caused by dominant negative mutations in signal transducer and activator of transcription (STAT) 3. The syndrome is characterized by infectious, immunologic, and non-immunologic manifestations and is associated with significant morbidity, mortality, and development of lymphomas. What has not yet been elucidated is the role of HSCT in the disease treatment...

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PMID: 32497403
DOI: 10.1111/petr.13699

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