Chronic Granulomatous Disease-Associated IBD Resolves and Does Not Adversely Impact Survival Following Allogeneic HCT
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ORIGINAL ARTICLE
Chronic Granulomatous Disease-Associated IBD Resolves and Does Not Adversely Impact Survival Following Allogeneic HCT Rebecca A. Marsh 1 & Jennifer W. Leiding 2 & Brent R. Logan 3 & Linda M. Griffith 4 & Danielle E. Arnold 5 & Elie Haddad 6 & E. Liana Falcone 7 & Ziyan Yin 3 & Kadam Patel 3 & Erin Arbuckle 8 & Jack J. Bleesing 1 & Kathleen E. Sullivan 5 & Jennifer Heimall 5 & Lauri M. Burroughs 9 & Suzanne Skoda-Smith 10 & Shanmuganathan Chandrakasan 11 & Lolie C. Yu 12 & Benjamin R. Oshrine 13 & Geoffrey D. E. Cuvelier 14 & Monica S. Thakar 15 & Karin Chen 16 & Pierre Teira 17 & Shalini Shenoy 18 & Rachel Phelan 19 & Lisa R. Forbes 20 & Deepak Chellapandian 21 & Blachy J. Dávila Saldaña 22 & Ami J. Shah 23 & Katja G. Weinacht 24 & Avni Joshi 25 & Farid Boulad 26 & Troy C. Quigg 27 & Christopher C. Dvorak 28 & Debi Grossman 29 & Troy Torgerson 30 & Pamela Graham 29 & Vinod Prasad 31 & Alan Knutsen 32 & Hey Chong 33 & Holly Miller 34 & M. Teresa de la Morena 35 & Kenneth DeSantes 36 & Morton J. Cowan 28 & Luigi D. Notarangelo 37 & Donald B. Kohn 38 & Elizabeth Stenger 39 & Sung-Yun Pai 40 & John M. Routes 41 & Jennifer M. Puck 28 & Neena Kapoor 42 & Michael A. Pulsipher 42 & Harry L. Malech 29 & Suhag Parikh 43 & Elizabeth M. Kang 29 & submitted on behalf of the Primary Immune Deficiency Treatment Consortium Received: 11 January 2019 / Accepted: 10 June 2019 # This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply 2019
Abstract Introduction Inflammatory bowel disease (IBD) affects approximately 1/3 of patients with chronic granulomatous disease (CGD). Comprehensive investigation of the effect of allogeneic hematopoietic cell transplantation (HCT) on CGD IBD and the impact of IBD on transplant outcomes is lacking. Methods We collected data retrospectively from 145 patients with CGD who had received allogeneic HCT at 26 Primary Immune Deficiency Treatment Consortium (PIDTC) centers between January 1, 2005 and June 30, 2016. Results Forty-nine CGD patients with IBD and 96 patients without IBD underwent allogeneic HCT. Eighty-nine percent of patients with IBD and 93% of patients without IBD engrafted (p = 0.476). Upper gastrointestinal acute GVHD occurred in 8.5% of patients with IBD and 3.5% of patients without IBD (p = 0.246). Lower gastrointestinal acute GVHD occurred in 10.6% of patients with IBD and 11.8% of patients without IBD (p = 0.845). The cumulative incidence of acute GVHD grades II–IV was 30% (CI 17–43%) in patients with IBD and 20% (CI 12–29%) in patients without IBD (p = 0.09). Five-year overall survival was equivalent for patients with and without IBD: 80% [CI 66–89%] and 83% [CI 72–90%], respectively (p = 0.689). All 33 surviving evaluable patients with a history of IBD experienced resolution of IBD by 2 years following allogeneic HCT.
Rebecca A. Marsh, Jennifer W. Leiding, Brent R. Logan, Harry Malech, Suhag Parikh, and Elizabeth M. Kang contributed equally to this work. Key Points • CGD-associated inflammatory bowel disea
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