Detection of Bronchiolitis Obliterans Syndrome Following Pediatric Hematopoietic Stem Cell Transplantation

Publication Date: June 18, 2024
Last Updated: September 27, 2024

Summary of Recommendations

Recommendation 1

We recommend pre-HSCT spirometry, static lung volumes, and diffusing capacity of the lungs for carbon monoxide (DLco) for children who can perform them.

(S, M )
620

Recommendation 2a

We suggest active surveillance rather than testing only symptomatic patients using spirometry and where feasible, static lung volumes, and DLco beginning at 3 months post-HSCT. (C, L )
620

Recommendation 2b

We suggest spirometry and where feasible static lung volumes and DLCO, be performed every 3 months in the first year post-HSCT and every 3 to 6 months in the second year post-HSCT in patients not at high risk of BOS.

(C, L )

Comment: More frequent testing may be indicated in those at high risk of pulmonary complications or with cGvHD in other organs.

620

Recommendation 2c

For long-term follow-up in asymptomatic patients, we suggest surveillance using spirometry and where feasible, static lung volumes and DLco every 6 months, between 2 and 3 years post-HSCT and yearly after 3 years lasting until 10 years post-HSCT. (C, L )
Comment: In patients with ongoing symptoms, more frequent (3-6 monthly) spirometry maybe necessary until stability in lung function testing has been demonstrated.
620

Recommendation 3a

At centers with adequate technical expertise to perform multiple breath washout (MBW), we suggest including MBW and spirometry as part of a pre-HSCT assessment of pulmonary function, or MBW alone if spirometry is not feasible. (C, L )
620

Recommendation 3b

At centers with adequate technical expertise to perform MBW, we suggest the use of post-HSCT MBW as part of the diagnostic evaluation of suspected BOS, either as a complementary tool to spirometry or alone if spirometry is not feasible. (C, VL )
620

Recommendation 4a

We suggest performing a chest computed tomography (CT) scan, with inspiratory and expiratory views, in all children prior to allogeneic HSCT. (C, L )
Comment: In situations where the clinical team identifies a low risk of pre-existing lung disease, it is reasonable to not perform a pre- CT scan. Additionally, a pre- CT scan does not need to be performed in patients with an ionizing-radiation sensitive condition (i.e., Fanconi anemia).
620

Recommendation 4b

We suggest performing a chest CT scan with inspiratory and expiratory views, in all children post allogeneic HSCT who develop obstructive lung function or in those children with clinical suspicion of BOS.

(C, L )
620

Recommendation 6

We suggest surgical lung biopsy in pediatric post-HSCT patients where BOS is suspected, but uncertainty regarding the diagnosis exists and the risks of biopsy are smaller than the risks of the uncertainty.

(C, L )

Comment: Uncertainty regarding the diagnosis exists when: A) clinical evidence (clinical background/CT scan/pulmonary function testing) is discordant; B) there is no alternate way to make the diagnosis; C) there is concern for an alternate/co-existing condition.

620

Recommendation Grading

Disclaimer

The information in this patient summary should not be used as a substitute for professional medical care or advice. Contact a health care provider if you have questions about your health.

Overview

Title

Detection of Bronchiolitis Obliterans Syndrome Following Pediatric Hematopoietic Stem Cell Transplantation

Authoring Organization

American Thoracic Society

Publication Month/Year

June 18, 2024

Last Updated Month/Year

October 4, 2024

Document Type

Guideline

Country of Publication

US

Document Objectives

Many children undergo allogeneic Hematopoietic Stem Cell Transplantation (HSCT) for the treatment of malignant and non-malignant conditions. Unfortunately, pulmonary complications occur frequently post-HSCT, with bronchiolitis obliterans syndrome (BOS) being the most common non-infectious pulmonary complication. Current international guidelines contain conflicting recommendations regarding post-HSCT surveillance for BOS, and a recent National Institutes of Health workshop highlighted the need for a standardized approach to post-HSCT monitoring. As such, this guideline provides an evidence-based approach to detection of post-HSCT BOS in children. This document provides an evidence-based approach to detection of post-HSCT BOS in children, while also highlighting considerations for implementation of each recommendation. Further, the document describes important areas for future research.

PICO Questions

  1. Should Pre-HSCT Screening Spirometry, Static Lung Volumes, and DLCO Be Performed in Pediatric Patients Who Will Undergo Allogeneic HSCT?

  2. Should Routine Surveillance Spirometry Be Performed Post–Allogeneic HSCT in Pediatric Patients?

  3. In Pediatric Patients Who Have Had Allogeneic HSCT, Should the Routine Surveillance of Lung Function Be Done Using Spirometry or a Combination of MBW and Spirometry?

  4. Should Pediatric Patients Post–Allogeneic HSCT Who Have Abnormal Surveillance Lung Function Assessment Be Investigated with a Chest CT Scan?

  5. Should Pediatric Patients Post–Allogeneic HSCT Who Have Abnormal Surveillance Lung Function Assessment Be Investigated with a BAL/Bronchoscopy?

  6. In Allogeneic HSCT Pediatric Patients with Suspected BO, Should Lung Biopsy Be Used to Diagnose BO?

Inclusion Criteria

Male, Female, Adolescent, Child, Infant

Health Care Settings

Ambulatory, Hospital, Outpatient

Intended Users

Nurse, nurse practitioner, physician, physician assistant

Scope

Assessment and screening, Management

Diseases/Conditions (MeSH)

D001989 - Bronchiolitis Obliterans, D000092122 - Bronchiolitis Obliterans Syndrome

Keywords

stem cell transplantation, bronchiolitis, HSCT, bronchiolitis obliterans syndrome

Source Citation

Shanthikumar S, Document S, Gower WA, Srinivasan S, Rayment JH, Robinson PD, Bracken J, Stone A, Das S, Barochia A, Charbek E, Tamae-Kakazu M, Reardon EE, Abts M, Blinman T, Calvo C, Cheng PC, Cole TS, Cooke KR, Davies SM, De A, Gross J, Mechinaud F, Sheshadri A, Siddaiah R, Teusink-Cross A, Towe CT, Walkup LL, Yanik GA, Bergeron A, Casey A, Deterding RR, Liptzin DR, Schultz KR, Iyer NP, Goldfarb S. Detection of Bronchiolitis Obliterans Syndrome Following Pediatric Hematopoietic Stem Cell Transplantation. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med. 2024 Jun 18. doi: 10.1164/rccm.202406-1117ST. Epub ahead of print. PMID: 38889365.