Rapid full engraftment and successful immune reconstitution after allogeneic hematopoietic stem cell transplantation with reduced intensity conditioning in Omenn syndrome.
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.
Gozdzik
Jolanta
J
Department of Transplantation, Polish-American Institute of Pediatrics, Jagiellonian University Medical College, Cracow, Poland. jgozdzik@cm-uj.krakow.pl
Czogala
Wojciech
W
Skoczen
Szymon
S
Krasowska-Kwiecien
Aleksandra
A
Wiecha
Oktawiusz
O
Mordel
Anna
A
Lesko
Ewa
E
Majka
Marcin
M
Kowalczyk
Danuta
D
Zembala
Marek
M
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
D000998:Antiviral Agents; D007166:Immunosuppressive Agents