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Ruxolitinib is an effective steroid sparing agent in children with steroid refractory/dependent bronchiolitis obliterans syndrome after allogenic hematopoietic cell transplantation

Bone Marrow Transplantation volume 54, pages 1158–1160 (2019)Cite this article

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Bronchiolitis obliterans syndrome (BOS), chronic graft versus host disease (cGVHD) of the lungs, confers a dismal prognosis [1, 2]. Steroids are the backbone therapy for BOS, but prolonged use is associated with increased mortality and is not recommended [2, 3]. However, there is no consensus therapy for patients who are steroid dependent or refractory (SR).

Ruxolitinib, a JAK1/2 inhibitor, exerts potent anti-inflammatory effects and is FDA approved to treat acute GVHD in adults. Ruxolitinib therapy for cGVHD in adults is also described [4], though reports of ruxolitinib use in children for GVHD are rare [5, 6]. We describe a single center experience of pediatric patients with SR-BOS treated with ruxolitinib.

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Acknowledgements

We are grateful for our funding support, Pedals for Pediatrics and the NIH NIAID.

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Author notes

  1. These authors contributed equally: Meera Subramaniam, Steven Margossian

Authors and Affiliations

  1. Boston Children’s/Dana Farber Cancer and Blood Disorders Center, Boston, MA, USA

    Michelle Schoettler, Christine Duncan, Leslie Lehmann, Elissa Furutani & Steven Margossian

  2. Department of Pulmonology, Boston Children’s Hospital, Boston, MA, USA

    Meera Subramaniam

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  1. Michelle Schoettler
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  2. Christine Duncan
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  3. Leslie Lehmann
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  4. Elissa Furutani
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  5. Meera Subramaniam
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  6. Steven Margossian
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Correspondence to Michelle Schoettler.

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Schoettler, M., Duncan, C., Lehmann, L. et al. Ruxolitinib is an effective steroid sparing agent in children with steroid refractory/dependent bronchiolitis obliterans syndrome after allogenic hematopoietic cell transplantation. Bone Marrow Transplant 54, 1158–1160 (2019). https://doi.org/10.1038/s41409-019-0450-3

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