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

Abstract

Background Bronchiolitis obliterans syndrome (BOS) is a life‐threatening respiratory complication of allogeneic hematopoietic stem cell transplantation (HSCT). Although pulmonary function testing is crucial for monitoring BOS, little information exists on the association of these test results with outcomes in children with BOS. Objectives The purpose of this study was to determine the correlation between changes in lung function after BOS diagnosis and long‐term outcomes. Methods A total of 428 children underwent allogeneic HSCT from January 2006 to December 2017 at Asan Medical Center. Twenty‐three (5.4%) were diagnosed with BOS after allogeneic HSCT, and their clinical data were reviewed. Twenty‐one subjects underwent regular pulmonary function testing for 24 months after BOS diagnosis. Results Among the 21 children with BOS, 8 died, 5 underwent lung transplantation (TPL), and 15 required oxygen (O2) therapy. The FEV1% predicted (pred), FVC% pred, and FEF25%–75% pred were 37.8 ± 12.7% (mean ± SD), 62.2 ± 16.2%, and 16.4 ± 9.6%, respectively, at the time of BOS diagnosis. Changes in the FEV1% pred were greater in the death and lung TPL groups (−24.8 ± 22.3%) than in the survival without lung TPL group (5.7 ± 21.8%) and greater in the O2 therapy (−19.4 ± 23.4%) group than in the group without O2 therapy (14.2 ± 20.0%) during the first 3 months after BOS diagnosis. Conclusion The change in FEV1 during the first 3 months after BOS diagnosis correlated with outcomes including survival, lung TPL, and O2 therapy. These results suggest that more active intervention in the first 3 months after BOS diagnosis may be needed to improve prognosis.

Pediatric pulmonology year in review 2021 and 2022: Physiology

“…Declining lung function is a defining characteristic in bronchiolitis obliterans syndrome (BOS), however the clinical implications of lung function changes in BOS are unknown. Jung et al 50 | 2207 into this issue, Hardie et al 51 performed a retrospective review of testing (CPET). Although the majority (64%) of patients reported dyspnea on exertion, pulmonary limitation during exercise was present in only 3% of the study population, and there was no correlation between CPET results and symptoms of dyspnea or chest pain on exertion.…”

“…Declining lung function is a defining characteristic in bronchiolitis obliterans syndrome (BOS), however the clinical implications of lung function changes in BOS are unknown. Jung et al 50 examined the relationship between lung function decline and clinical outcomes in patients with BOS following allogenic hematopoetic stem cell transplantation. They found that a rapid decline in FEV 1 in the first 3 months after BOS diagnosis correlated with poor clinical outcomes, including death, need for lung transplantation, or oxygen therapy.…”

Pediatric pulmonology 2021 year in review: Rare and diffuse lung disease

“…Mean decline in forced expiratory volume in one second (FEV 1 ) (1.07% predicted and 0.075 in Z ‐score annually) was noted, with preservation of FVC, over an average follow‐up period of 8 years, suggesting ongoing lung growth despite persistent obstruction. Jung et al 48 reported on 21 patients with BO syndrome (patients with CT and PFT findings consistent with BO but lacking a biopsy) from a cohort of 428 who received HSCT from 2006 through 2017 at Asan Medical Center in Seoul. They also noted a high degree of morbidity and mortality with eight deaths (38%), and five (24%) progressing to lung transplantation.…”

Pulmonary complications after hematopoietic stem cell transplantation in children: a functional and tomographic evaluation

“…(1)(2)(3)(4) Testes de função pulmonar (TFPs) podem ajudar a identificar o nível de deterioração no período pós-TCTH. (7) Embora a tomografia computadorizada (TC) seja o método de escolha para detectar anormalidades pulmonares, os achados tomográficos geralmente são inespecíficos e requerem correlações temporais baseadas no estado imune do paciente. (5,6,8) Foi realizada uma revisão retrospectiva de todos os pacientes menores de 14 anos que receberam TCTH em um centro de referência no período de 2013 a A TC de tórax foi realizada em 75 (46,6%) pacientes no acompanhamento pós-transplante, dos quais 61 (81,3%) apresentavam anormalidades.…”

“…(10) Jung et al (2021) mostraram que alterações no VEF1 nos primeiros 3 meses após o diagnóstico de BO impactaram desfechos como óbito e transplante de pulmão, indicando a necessidade de monitoramento e intervenções mais precoces para alterar os indicadores de sobrevida. (7) As alterações tomográficas de tórax encontradas foram destacadas em revisões anteriores, principalmente 2/3…”