Long‐term pulmonary function testing in pediatric bronchiolitis obliterans syndrome after hematopoietic stem cell transplantation

Abstract

Rationale Bronchiolitis obliterans syndrome (BOS) is a severe, chronic inflammation of the airways leading to an obstruction of the bronchioles. So far, there are only a few studies looking at the long‐term development of pulmonary impairment in children with BOS. Objective The objective of this study was to investigate the incidence and long‐term outcome of BOS in children who underwent allogeneic hematopoietic stem cell transplantation (HSCT). Methods Medical charts of 526 children undergoing HSCT in Frankfurt/Main, Germany between 2000 and 2017 were analyzed retrospectively and as a result, 14 patients with BOS were identified. A total of 271 lung functions (spirometry and body plethysmography), 26 lung clearance indices (LCI), and 46 chest high‐resolution computed tomography (HRCT) of these 14 patients with BOS were evaluated. Results Fourteen patients suffered from BOS after HSCT (2.7%), whereby three distinctive patterns of lung function impairment were observed: three out of 14 patients showed a progressive lung function decline; two died and one received a lung transplant. In five out of 14 patients with BOS persisted with a severe obstructive and secondarily restrictive pattern in lung function (forced vital capacity [FVC] < 60%, forced expiratory volume in 1 second [FEV1] < 50%, and FEV1/FVC < 0.7) and increased LCI (11.67‐20.9), six out of 14 patients recovered completely after moderate lung function impairment and signs of BOS on HRCT. Long‐term FVC in absolute numbers was increased indicating that the children still have lung growth. Conclusion Our results showed that the incidence of BOS in children is low. BOS was associated with high mortality and may lead to persistent obstructive lung disease; although, lung growth continued to exist.

Follow-up on the therapeutic effects of a budesonide, azithromycin, montelukast, and acetylcysteine (BAMA) regimen in children with post-infectious bronchiolitis obliterans

“…Bronchiolitis obliterans (BO) is a rare chronic lung disease that can cause irreversible obstruction or occlusion of small airways and mainly results from a severe insult to the lower respiratory tract (1). There have been many reports of BO developing after bone marrow transplantation (BMT), haematopoietic stem cell transplantation (HSCT), or lung transplantation (2)(3)(4)(5)(6)(7). Additionally, the…”

The association of lung function changes with outcomes in children with bronchiolitis obliterans syndrome after hematopoietic stem cell transplantation

“…BOS is characterized by irreversible small airway obstruction caused by epithelial injury, subepithelial inflammation, and fibrosis of small airways 2 . The prevalence of BOS has been reported to be 2.0%–6.5% in allogeneic HSCT recipients in adult cohorts 3–5 and 2.0%–2.7% in pediatric cohorts 6,7 . The prognosis of children with BOS after HSCT is poor; the 5‐year survival rate is 45.0%–59.0% 8,9 .…”

Identification of pediatric bronchiolitis obliterans syndrome posthematopoietic stem cell transplantation; surveillance is the key

“…We read with great interest the article by Walther et al 1 regarding long term outcomes of bronchiolitis obliterans syndrome (BOS) in children following haematopoietic stem cell transplantation (HSCT). The authors are to be commended on their study as it is an important contribution to a field where there is a relative paucity of evidence.…”

“…In summary, the article by Walther et al 1 However, the lack of a formal surveillance program and likely resultant underestimation of BOS cases is a significant limitation that should be acknowledged before efforts are made to prevent or attenuate lung function decline in BOS.…”

“…The authors declare that there are no conflict of interests. Shivanthan Shanthikumar FRACP 1,2,3 Liam Welsh PhD 1,2 Nicole Westrupp MS 1,2 Theresa Cole PhD 3,4 Katherine B. Frayman FRACP 1,2,3…”