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Review

Bronchiolitis obliterans syndrome in adults after allogeneic stem cell transplantation-pathophysiology, diagnostics and treatment

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Pages 553-569 | Received 07 Jun 2016, Accepted 22 Dec 2016, Published online: 17 Jan 2017
 

ABSTRACT

Introduction: Transplant-related complications are common after allogeneic hematopoietic stem cell transplantation (allo-HSCT), including graft versus host disease (GVHD). The lungs are frequently affected during the course of allo-HSCT, and among the non-infectious pulmonary complications bronchiolitis obliterans syndrome (BOS) is the most common and considered the only diagnostic manifestation of pulmonary GVHD. BOS is an irreversible obstructive disease that affects the terminal bronchioles, and it is associated with high morbidity and mortality rates.

Area covered: We discuss the features of chronic GVHD, including the pathophysiological and cytokine-mediated alteration in BOS. Early treatment, before structural and irreversible changes have occurred, is crucial to reduce disease morbidity and mortality. This is challenging, given the unspecific symptoms of early stage disease and the complexity of the disease pathophysiology, obstructing both the diagnostic workup and the initiation of treatment. We highlight the main issues regarding diagnostic challenges, and we discuss the treatment options with a focus on new therapeutic options and modalities.

Expert commentary: BOS is one of the most serious late complications after allo-HSCT and remains a diagnostic and therapeutic challenge. Thus, new and more effective therapeutic alternatives are strongly warranted.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Additional information

Funding

The study was supported by Øyvinn Mølbach-Pedersen’s foundation, Helse Vest and the Norwegian Cancer Society.

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