Rapid full engraftment and successful immune reconstitution after allogeneic hematopoietic stem cell transplantation with reduced intensity conditioning in Omenn syndrome

Autor: Anna Mordel, Marcin Majka, Ewa Lesko, Wojciech Czogała, Danuta Kowalczyk, Marek Zembala, Oktawiusz Wiecha, Szymon Skoczeń, Aleksandra Krasowska-Kwiecień, Jolanta Gozdzik
Rok vydání: 2009
Předmět:
Zdroj: Pediatric Transplantation. 13:760-765
ISSN: 1399-3046
1397-3142
DOI: 10.1111/j.1399-3046.2008.01020.x
Popis: OS is a variant of SCID characterized by generalized erythroderma, alopecia, eosinophilia, and elevated IgE levels. It is fatal unless treated with allogeneic HSCT, which is the only curative approach. However, treatment related complications and graft rejection are major obstacles to the success of treatment. In this report, we describe a patient with OS, complicated by prolonged cytomegalovirus infection, successfully treated by reduced intensity conditioning allogeneic HSCT from sibling donor.
Databáze: OpenAIRE