Gülay Sezgin, Turkey
Çukurova University Medical School Division of Pediatric Oncology/BMT UnitPresenter of 1 Presentation
STEM CELL TRANSPLANTATION IN PRIMARY IMMUNODEFICIENCIES:SINGLE CENTER EXPERIENCE IN SOUTHERN TURKEY
Abstract
Background and Aims
Stem cell transplantation is the only cure for most of the PIDs. As an newly established transplant unit we have transplanted 18 patients with primary immunodeficiencies between 2013-2019.
Methods
Eighteen patients were transplanted between 2013-2019. Three of them were Syrian refugees.There were 14 boys 6 girls. The median age was 5 years (min 1 max 12 years) There were 11 MSD, 4 matched MUD, 4 one allele mismatch MUD and one haploidentical transplants. Two patients were transplanted twice due to graft failure. Their diagnoses were as follows; 3 CGD, 2 MHC Class II deficiency, 1 WAS, 1 LAD I deficiency, 1 Kostman syndrome, 8 SCIDs, 1 cartilage hair dysplasia with immunodeficiency, 1 Dock 8 deficiency.
Results
12 patients are engrafted and alive. Of the 6 patients who died,one had graft failure and went for second transplant. All patients died due to interfering infections and respiratory distress posttransplant.
Conclusions
There has been a great improvement in treating PID with SCT with a greater understanding of the genetics and immunobiology of these diseases and also facilitating the matching of donor type and conditioning regimens.