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Diagnosis and treatment of bronchiolitis obliterans syndrome accessible universally

Bone Marrow Transplantation volume 54, pages 383–392 (2019)Cite this article

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Abstract

The incidence of bronchiolitis obliterans syndrome (BOS), a devastating manifestation of chronic graft-versus-host-disease, may rise globally due to steady increases in utilization of allogeneic hematopoietic cell transplantation (HCT). Though some advances have occurred in the past decade regarding understanding of the pathogenesis, diagnosis and treatment of BOS, the overall mortality and morbidity remain very high. We sought to determine the current diagnostic and therapeutic challenges, which can potentially hinder optimal management of BOS both in developed and developing countries. We performed a comprehensive systematic review of both modern diagnostic modalities and treatments and then assessed which of them would be universally accessible. The 2014 National Institutes of Health chronic GVHD criteria remains the gold standard tool for diagnosing BOS. Important elements of treatment involve early and accurate detection, as well as utilizing the treatment modalities with known (but variable efficacy) e.g. fluticasone-azithromycin-montelukast [FAM] combination, etanercept, extra-corporeal photopheresis [ECP], lung transplantation, and prompt treatment of complications including infections in sufferers of BOS. Our results indicate that optimum diagnostic tools are not readily available in some parts of the world for early detection, which include a lack of CT scanners, unavailability of pulmonary function testing tools, absence of sub-specialists, lack of certain effective treatments and late referral for lung transplant. We present a systematic review of current literature along with recommendations for available therapies to guide practitioners to optimize the long-term outcomes in HCT survivors regardless of access to experts and expensive therapies.

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Acknowledgements

Author contribution

AH and SKH wrote the first draft of the manuscript. All authors contributed substantially to the conception, acquisition, analysis, and interpretation of the data for the work and approved the final approval of the version to be published.

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Authors and Affiliations

  1. Division of Hematology and Stem Cell Transplant, King Faisal Specialist Hospital, Riyadh, Saudi Arabia

    Anas Hakim & Shahrukh K. Hashmi

  2. Department of Oncology, Johns Hopkins School of Medicine, Baltimore, MD, USA

    Kenneth R. Cooke

  3. Experimental Transplantation and Immunology Branch, NIH, Bethesda, MD, USA

    Steven Z. Pavletic

  4. Division of Pulmonology, Department of Medicine, King Faisal Hospital, Riyadh, Saudi Arabia

    Mohammad Khalid

  5. Center for Cancer and Blood Disorders, Children’s National Health System, Washington, DC, USA

    Kirsten M. Williams

  6. Blood and Marrow Transplant Division, Transplant Center, Mayo Clinic, Rochester, USA

    Shahrukh K. Hashmi

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  1. Anas Hakim
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Correspondence to Shahrukh K. Hashmi.

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Hakim, A., Cooke, K.R., Pavletic, S.Z. et al. Diagnosis and treatment of bronchiolitis obliterans syndrome accessible universally. Bone Marrow Transplant 54, 383–392 (2019). https://doi.org/10.1038/s41409-018-0266-6

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