Successful engraftment following unrelated donor transplant in an alloimmunized patient with Kostmann syndrome.
Severe chronic neutropenia (SCN) is characterized by severe recurrent bacterial infections during infancy. Blood or marrow transplantation (BMT) is the only curative option for patients with refractory disease. This report describes the case of a 4-year-old girl with refractory SCN, who received a bone marrow transplant from a highly matched donor after becoming HLA sensitized to multiple granulocyte transfusions. She is clinically well with normal blood counts and stable mixed chimerism 3 years after BMT. She experienced no graft rejection or graft versus host disease.
University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania 15213, USA.
Scott N
SN
Myers
Adriana
A
Zeevi
G Phil
GP
Zorich
Gina
G
Pillage
Joan
J
Martel
Rakesh K
RK
Goyal
D016026:Bone Marrow Transplantation / Q000276:immunology*; D002675:Child, Preschool; D002908:Chronic Disease; D005260:Female; D006085:Graft Survival; D006801:Humans; D007114:Immunization; D009503:Neutropenia / Q000235:genetics / Q000628:therapy*; D016879:Salvage Therapy; D013577:Syndrome; D014019:Tissue Donors; D014181:Transplantation Immunology; D014184:Transplantation, Homologous