Abstract
Bronchiolitis obliterans syndrome (BOS) is a life-threatening pulmonary manifestation of chronic graft versus host disease (cGVHD) post-allogeneic hematopoietic stem cell transplantation (HSCT), without clear standard of care. This study included 30 patients undergoing an allogeneic HSCT for a hematological malignancy and the outcomes with post-HSCT BOS treated with ruxolitinib as a salvage treatment were reviewed. After a median duration of ruxolitinib therapy of 9.25 (1.5–27) months, the best overall response (BOR) rate was 66.7%: three patients (10.0%) achieved complete remission, and 17 (56.7%) achieved partial remission. The median time from initiation of ruxolitinib to achieve the best responses was 3 months. Since initiating ruxolitinib, forced expiratory volume in 1 s of predicted (FEV1%pred) slightly increased after 3 and 6 months compared with measurements before ruxolitinib in responders. Only FEV1%pred mild decline before ruxolitinib with a ratio ≤15% was an independent predictor to achieve a response to ruxolitinib. Eleven patients (36.7%) had severe pulmonary infection of ≥3 grade. Following a median follow-up of 318 days after ruxolitinib, the 2-years incidence of nonrelapse mortality and 2-years overall survival rate after ruxolitinib among patients with BOS was 25.1 and 62.6%, respectively. Ruxolitinib is a promising treatment option to improve the prognosis of post-HSCT BOS.
Highlights
Bronchiolitis obliterans syndrome (BOS) is a pulmonary fibroproliferative disorder characterized by irreversible narrowing and obliteration of the small airways. (Barker et al, 2014)
The inclusion criteria were as follows: 1) patients aged ≥14 years; 2) diagnosed with BOS related to chronic graft versus host disease (cGVHD) according to the criteria of the National Institute of Health (NIH) consensus; 3) treated with systemic glucocorticosteroids (GCSs) in combination with therapy using inhaled corticosteroids fluticasone, azithromycin, and montelukast (FAM) with/ without calcineurin inhibitors (CNIs), and had worsening lung function, which was defined as a decline in forced expiratory volume in 1 s of predicted (FEV1%pred) from baseline by 5% or more; and 4) treated with ruxolitinib as a salvage treatment for at least 30 days
The present study added to a growing body of evidence for the use of ruxolitinib in SR-BOS related to cGVHD
Summary
Bronchiolitis obliterans syndrome (BOS) is a pulmonary fibroproliferative disorder characterized by irreversible narrowing and obliteration of the small airways. (Barker et al, 2014). In the setting of allogeneic hematopoietic stem cell transplantation (allo-HSCT), BOS is the most common pulmonary manifestation of moderate/severe chronic graft versus host disease (cGVHD), and the overall prevalence of BOS is up to 14% among patients who develop cGVHD (Au et al, 2011). Combination of inhaled corticosteroids and bronchodilators, azithromycin and montelukast early in BOS therapy could reduce total corticosteroids exposure (Bergeron et al, 2015; Kim et al, 2016; Williams et al, 2016). No consensus has been reached regarding the optimal therapy for BOS because of suboptimal responses to medical therapies, and effective therapeutic strategies are needed to stabilize lung function, reduce corticosteroid dosing, and improve quality of life for patients with BOS after HSCT
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