title: Rapid full engraftment and successful immune reconstitution after allogeneic hematopoietic stem cell transplantation with reduced intensity conditioning in Omenn syndrome.
abstract: 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.
authors.affiliation: Department of Transplantation, Polish-American Institute of Pediatrics, Jagiellonian University Medical College, Cracow, Poland. jgozdzik@cm-uj.krakow.pl
authors.forename: Jolanta
authors.initials: J
authors.lastname: Gozdzik
authors.forename: Wojciech
authors.initials: W
authors.lastname: Czogala
authors.forename: Szymon
authors.initials: S
authors.lastname: Skoczen
authors.forename: Aleksandra
authors.initials: A
authors.lastname: Krasowska-Kwiecien
authors.forename: Oktawiusz
authors.initials: O
authors.lastname: Wiecha
authors.forename: Anna
authors.initials: A
authors.lastname: Mordel
authors.forename: Ewa
authors.initials: E
authors.lastname: Lesko
authors.forename: Marcin
authors.initials: M
authors.lastname: Majka
authors.forename: Danuta
authors.initials: D
authors.lastname: Kowalczyk
authors.forename: Marek
authors.initials: M
authors.lastname: Zembala
mesh_terms: D000998:Antiviral Agents / Q000627:therapeutic use; D016026:Bone Marrow Transplantation / Q000379:methods*; D003586:Cytomegalovirus Infections / Q000209:etiology / Q000628:therapy; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects / Q000379:methods*; D006801:Humans; D007107:Immune System; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D008214:Lymphocytes / Q000166:cytology; D008297:Male; D016511:Severe Combined Immunodeficiency / Q000150:complications / Q000628:therapy*; D035781:Siblings; D019172:Transplantation Conditioning / Q000379:methods*; D014184:Transplantation, Homologous / Q000379:methods; D016896:Treatment Outcome
chemical_list: D000998:Antiviral Agents; D007166:Immunosuppressive Agents