title: Outcome of boost haemopoietic stem cell transplant for decreased donor chimerism or graft dysfunction in primary immunodeficiency.
abstract: Haemopoietic stem cell transplants (HSCT) cure increasing numbers of primary immunodeficiencies (PID): residual recipient T-cell function increases risk of incomplete or decreasing immune reconstitution, which may resolve following a second, unconditioned, infusion from the same donor (boost infusion). We assessed the outcome of 20 boost infusions in 19/139 patients transplanted for PID patients at our centre since 1987. Boost infusion was given 64-1226 days after the original HSCT. Follow-up was 4-124 months. In all, 12 of 19 patients cleared viral infection (6), or showed sustained increase in donor chimerism, T- and B-cell numbers and function, or other markers (6). In 7/12 patients, immunoglobulin replacement has been discontinued. Four were partially successful with stable low-level chimerism (two patients) or improved T-cell function, but not B cell function (two patients). Four failed with no change in donor chimerism or cell number. No significant association with donor source, T-cell depletion, conditioning regimen, boost infusion stem cell dose or time from original HSCT to boost was found. One patient developed grade III acute graft-versus-host disease despite cyclosporine, and one developed severe pneumonitis; both have recovered. Boost infusion was successful or partially successful in 84% of patients. The risk of adverse effects is low.
authors.affiliation: Department of Paediatric Immunology, University of Newcastle upon Tyne, Newcastle General Hospital, NHS Trust, Newcastle upon Tyne, UK.
authors.forename: M A
authors.initials: MA
authors.lastname: Slatter
authors.forename: A
authors.initials: A
authors.lastname: Bhattacharya
authors.forename: M
authors.initials: M
authors.lastname: Abinun
authors.forename: T J
authors.initials: TJ
authors.lastname: Flood
authors.forename: A J
authors.initials: AJ
authors.lastname: Cant
authors.forename: A R
authors.initials: AR
authors.lastname: Gennery
mesh_terms: D005500:Follow-Up Studies; D006085:Graft Survival; D006086:Graft vs Host Disease / Q000209:etiology; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects / Q000379:methods*; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000628:therapy*; D008214:Lymphocytes / Q000166:cytology / Q000502:physiology; D012189:Retrospective Studies; D018183:Transplantation Chimera; D016896:Treatment Outcome; D014777:Virus Diseases / Q000628:therapy