title: Complications of Reduced Intensity Conditioning HSCT for XIAP Deficiency (Alloimmune Cytopenias and HLH) Successfully Managed With Donor Lymphocyte Infusion.
abstract: X-linked inhibitor of apoptosis protein deficiency is a rare illness and although stem cell transplant is curative, full intensity conditioning is associated with high mortality rates. We describe a child with unusual complications associated with residual host lymphocytes following reduced intensity stem cell transplant. Recipient derived, donor directed, antigranulocyte antibodies led to life-threatening and prolonged neutropenia and residual recipient lymphocytes reestablished hemophagocytic lymphohistiocytosis after withdrawal of immune suppression despite high levels of whole blood chimerism. Hemophagocytic lymphohistiocytosis was abolished following specific improvement in donor T-cell chimerism after donor lymphocyte infusions, and alloimmune cytopenias were no longer evident.
authors.affiliation: Departments of *Paediatric Immunology †Transplantation Laboratory ‡Paediatric Haematology, Royal Manchester Children's Hospital, Central Manchester Foundation Trust NHS Hospitals, Manchester, UK.
authors.forename: Anu S
authors.initials: AS
authors.lastname: Varghese
authors.forename: Helena
authors.initials: H
authors.lastname: Lee
authors.forename: Denise
authors.initials: D
authors.lastname: Bonney
authors.forename: Stephen
authors.initials: S
authors.lastname: Hughes
authors.forename: Robert
authors.initials: R
authors.lastname: Wynn
mesh_terms: D000293:Adolescent; D003131:Combined Modality Therapy; D040181:Genetic Diseases, X-Linked / Q000150:complications* / Q000473:pathology / Q000628:therapy*; D006086:Graft vs Host Disease / Q000209:etiology / Q000473:pathology / Q000517:prevention & control*; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D008214:Lymphocytes / Q000166:cytology*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology / Q000473:pathology / Q000517:prevention & control; D008232:Lymphoproliferative Disorders / Q000150:complications* / Q000473:pathology / Q000628:therapy*; D008297:Male; D019653:Myeloablative Agonists / Q000627:therapeutic use; D010198:Pancytopenia / Q000209:etiology / Q000473:pathology / Q000517:prevention & control; D011379:Prognosis; D018183:Transplantation Chimera; D019172:Transplantation Conditioning / Q000009:adverse effects*; D014184:Transplantation, Homologous; D051636:X-Linked Inhibitor of Apoptosis Protein / Q000172:deficiency* / Q000235:genetics
chemical_list: D019653:Myeloablative Agonists; D051636:X-Linked Inhibitor of Apoptosis Protein; C496866:XIAP protein, human