Abstract：
:There have been no studies on patient outcome after allogeneic hematopoietic cell transplantation (HCT) in patients with X-linked inhibitor of apoptosis (XIAP) deficiency. To estimate the success of HCT, we conducted an international survey of transplantation outcomes. Data were reported for 19 patients. Seven patients received busulfan-containing myeloablative conditioning (MAC) regimens. Eleven patients underwent reduced intensity conditioning (RIC) regimens predominantly consisting of alemtuzumab, fludarabine, and melphalan. One patient received an intermediate-intensity regimen. Survival was poor in the MAC group, with only 1 patient surviving (14%). Most deaths were from transplantation-related toxicities, including venoocclusive disease and pulmonary hemorrhage. Of the 11 patients who received RIC, 6 are currently surviving at a median of 570 days after HCT (55%). Preparative regimen and HLH activity affected outcomes, and of RIC patients reported to be in remission from HLH, survival is 86% (P = .03). We conclude that MAC regimens should not be used for patients with XIAP deficiency. It is possible that the loss of XIAP and its antiapoptotic functions contributes to the high incidence of toxicities observed with MAC regimens. RIC regimens should be pursued with caution and, if possible, efforts should be made to ensure HLH remission before HCT in these patients.
journal_name
Bloodjournal_title
Bloodauthors
Marsh RA,Rao K,Satwani P,Lehmberg K,Müller I,Li D,Kim MO,Fischer A,Latour S,Sedlacek P,Barlogis V,Hamamoto K,Kanegane H,Milanovich S,Margolis DA,Dimmock D,Casper J,Douglas DN,Amrolia PJ,Veys P,Kumar AR,Jordan MBdoi
10.1182/blood-2012-06-432500subject
Has Abstractpub_date
2013-02-07 00:00:00pages
877-83issue
6eissn
0006-4971issn
1528-0020pii
blood-2012-06-432500journal_volume
121pub_type
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