J Clin Immunol (2017) 37:511–513 DOI 10.1007/s10875-017-0413-7 LETTER TO EDITOR IFNɣ Block, Treosulfan Conditioning and αβ T Cell Deplete PBSCT for XIAP-Deficient HLH 1 1 1 Ciara O’Rafferty & Mark Velangi & Sarah Lawson & 1 1 Prashant Hiwarkar & Jayashree Motwani Received: 17 April 2017 /Accepted: 14 June 2017 /Published online: 21 June 2017 Springer Science+Business Media, LLC 2017 The outcomes of hematopoietic stem cell transplantation The patient was commenced on the HLH 2004 protocol on (HSCT) for refractory hemophagocytic lymphohistiocytosis day ten of life (twice weekly etoposide, dexamethasone and (HLH) in X-linked inhibitor of apoptosis protein (XIAP) de- cyclosporin). Inflammatory markers showed a slow partial ficiency are poor . Amongst other functions, XIAP regu- response to initial therapy but on steroid taper, fevers re- emerged and ferritin rapidly increased. HLH 2004 protocol lates apoptosis in hepatocytes and other cells and its deficien- cy may result in increased sensitivity to chemotherapy. A high was complicated by Staphylococcus epidermidis bacteremia, rate of transplant-related mortality (TRM) is observed follow- Enterococcus growth from urine and respiratory syncytial vi- ing both myeloablative and reduced-intensity conditioning rus infection all of which responded promptly to antimicrobial regimens. Treatment-related mortality is attributed to uncon- treatment.
Journal of Clinical Immunology – Springer Journals
Published: Jun 21, 2017
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