Bronchiolitis obliterans syndrome after allogeneic hematopoietic SCT: phenotypes and prognosis

Abstract

1Bronchiolitis obliterans syndrome (BOS) after allogeneic hematopoietic SCT (HSCT) is recognized as a new-onset obstructive lung defect (OLD) in pulmonary function testing and is related to pulmonary chronic GVHD. Little is known about the different phenotypes of patients with BOS and their outcomes. We reviewed the data of all allogeneic HSCT recipients referred to our pulmonary department for a non-infectious bronchial disease between 1999 and 2010. We identified 103 patients (BOS (n ¼ 77), asthma (n ¼ 11) and chronic bronchitis (n ¼ 15)). In patients with BOS, we identified two functional phenotypes: a typical OLD, that is, forced expiratory volume in 1 s (FEV1)/forced vital capacity (FVC) ratio o0.7 (n ¼ 53), and an atypical OLD with a concomitant decrease in the FEV1 o80% and FVC o80% predicted with a normal total lung capacity (n ¼ 24). The typical OLD was characterized by more severe FEV1 and fewer centrilobular nodules on the computed tomography scan. The FEV1 was not significantly affected during the follow-up, regardless of the phenotype. In addition to acute and extensive chronic GVHD, only the occurrence of BOS soon after transplantation and the intentional treatment of BOS with steroids were associated with a poor survival. The determination of patient subgroups should be explored to improve the management of this condition.

Short-Term Variation of Lung Function and Airway Inflammation in Children and Adolescents with Bronchiolitis Obliterans

“…After a severe insult to the lower airways, a chronic inflammatory process leads to an inflammatory neutrophil response leading to partial or complete and progressive occlusion of the lumens of terminal and respiratory bronchioles [1]. The onset of the disease is generally seen in young children after a lower respiratory tract infection [2,3] and as graft versus host disease after transplantation [4,5]. Interestingly, BO patients of different aetiologies share key pathological findings suggesting that bronchiolitis obliterans could be the final common response pattern to different injuries to the lower respiratory tract.…”

Small airways diseases, excluding asthma and COPD: an overview

“…BOS after HSCT is associated with reduced survival [132], which is poorer when BOS occurs soon after transplantation [149]. Bronchoalveolar lavage shows either lymphocytic alveolitis or neutrophilia, and is not required for diagnosis [150].…”

“…An increase in systemic immunosuppression (including oral steroids) may increase rates of severe, potentially lethal, infections and should not be advised [149]. Inhaled steroids and long-acting bronchodilators may improve lung function and pulmonary symptoms, and have very limited side-effects [153][154][155].…”

Biopsy-Verified Bronchiolitis Obliterans and Other Noninfectious Lung Pathologies after Allogeneic Hematopoietic Stem Cell Transplantation

“…However, several modifications of the NIH BOS criteria have been applied in different studies [4,5,7,8,15,17,18,[42][43][44][45][46][47], which makes comparison difficult and indicates a need for a more simple and widely accepted classification.…”

“…Lung biopsy is the gold standard for diagnosis but is invasive and not widely used in the daily clinical routine. Instead, multiple diagnostic criteria based on clinical disease manifestations have been applied, which may partly explain the variability in the reported incidence (1.2% to 14.6%) [3][4][5][6][7][8][9][10][11][12][13][14] and mortality (27% to 90%) [3][4][5]7,[10][11][12][13]. To improve the basis for comparative research without running the risks associated with lung biopsy, in 2005 the National Institutes of Health (NIH) published criteria for BO syndrome (BOS), a clinical equivalent of biopsy-verified BO based on pulmonary function (PF) tests and radiology [2].…”