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- W2016105105 abstract "We performed a retrospective analysis on the outcome of 135 transplants for primary immunodeficiency syndromes in Australia and New Zealand Children's Haematology Oncology Group (ANZCHOG) transplant centres between 1992 and 2008. The most common conditions transplanted were Severe Combined Immunodeficiency (SCID) (48%), Wiskott-Aldrich Syndrome (WAS) (20%), and Chronic Granulomatous Disease (CGD) (12%). Median age at time of transplant was 1 year (range 0-15). Median follow up was 72 months. Stem cell source was matched sibling in 25%, parent in 30%, and unrelated donor in 45%. 55% of unrelated grafts were cord blood, the majority having at least one HLA mismatch. 57% received myeloablative conditioning (MAC), 25% received reduced intensity conditioning (RIC), and 17% had no conditioning. 48% had in-vivo T-cell depletion using ATG or Campath, and 30% had ex-vivo T-cell depletion. 18 patients failed to engraft, 15 engrafted following second transplant, 3 following third transplant, and 11 of the 18 remain long term survivors. Median time to neutrophil and platelet engraftment was 16 and 30 days respectively, with a cumulative incidence (CI) at day +42 of 86% and 63% respectively. The CI of aGVHD grade II-IV and III-IV at day +100 was 25% and 10% respectively. CI of cGVHD at 1 year was 16%. Transplant related mortality (TRM) was 10% at day +100, with infection, interstitial pneumonitis and GVHD the most common primary cause of death. 5 year overall survival (OS) was 72% for the cohort. Disease specific 5 year OS were SCID 70%, WAS 81% and CGD 69%. Multivariate analysis demonstrated having a transplant performed after 1998 significantly decreased the risk of aGvHD, but did not impact on overall survival. In contrast, development of interstitial pneumonitis, CMV infection/viraemia, aGVHD, and use of a mismatched family donor were independent variables that significantly decreased OS. Of interest, type of conditioning, GVHD prophylaxis, and T cell depletion did not significantly impact OS. In conclusion, this large series supports the use of transplantation as curative therapy for a range of primary immunodeficiency syndromes." @default.
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- W2016105105 date "2012-02-01" @default.
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- W2016105105 title "Haematopoietic Stem Cell Transplantation Outcomes in Primary Immunodeficiency: A Report from ANZCHOG and ABMTRR" @default.
- W2016105105 doi "https://doi.org/10.1016/j.bbmt.2011.12.259" @default.
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