0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Grand Rounds | Clinician's Corner

Bronchiolitis Obliterans After Allogeneic Hematopoietic Stem Cell Transplantation

Kirsten M. Williams, MD; Jason W. Chien, MD, MS; Mark T. Gladwin, MD; Steven Z. Pavletic, MD
JAMA. 2009;302(3):306-314. doi:10.1001/jama.2009.1018.
Text Size: A A A
Published online

With improvements in supportive care, both long-term survival following allogeneic hematopoietic stem cell transplantations (HSCTs) and the indications for this procedure have increased. As a result, the number of patients living with long-term toxic effects due to HSCT has increased. A once rare condition of the donor immune cells attacking healthy host tissues, termed chronic graft-vs-host disease, has become a more common phenomenon. When chronic graft-vs-host disease affects the lung tissue, bronchiolitis obliterans syndrome ensues. Recent data suggest that bronchiolitis obliterans syndrome may affect up to 6% of HSCT recipients and dramatically alters survival, with overall survival of only 13% at 5 years. These statistics have not improved since the first presentation of this disease over 20 years ago. Challenges to the progress of medical management of bronchiolitis obliterans syndrome include difficulties and delays in diagnosis and a paucity of data on pathogenesis to direct new therapies. This article critically evaluates the current diagnostic criteria for bronchiolitis obliterans syndrome and reviews the epidemiology, pathogenesis, and available treatments. Improvements in survival will likely require early disease recognition, allowing for therapeutic modulation of disease prior to the development of irreversible airway obliteration.

Figures in this Article

Sign in

Create a free personal account to sign up for alerts, share articles, and more.

Purchase Options

• Buy this article
• Subscribe to the journal

First Page Preview

View Large
First page PDF preview

Figures

Place holder to copy figure label and caption
Figure 1. Pulmonary Function Test of FEV1 Over Time After Hematopoietic Stem Cell Transplantation
Graphic Jump Location

FEV1 indicates forced expiratory volume in the first second of expiration.

Place holder to copy figure label and caption
Figure 2. Biopsy of Case Patient's Lung Tissue
Graphic Jump Location

A, Obliteration of bronchiolar lumen (hematoxylin-eosin, original magnification ×400). B, Inflammation between the epithelium and the smooth muscle (hematoxylin-eosin, original magnification ×400).

Place holder to copy figure label and caption
Figure 3. Hypothetical Spirogram and Flow Volume Curves for Bronchiolitis Obliterans Syndrome
Graphic Jump Location

The pulmonary function test pattern shown for patients with scleroderma and obstructive lung disease has been observed in patients with sclerotic graft-vs-host disease. FEV1 indicates forced expiratory volume in the first second of expiration; FVC, forced vital capacity; RV, residual volume; SVC, slow vital capacity; TLC, total lung capacity.

Place holder to copy figure label and caption
Figure 4. Chest Computed Tomography Scans for Bronchiolitis Obliterans (BO) Syndrome
Graphic Jump Location

A, Arrowheads indicate areas of air trapping. B, Dark areas show large areas of air trapping. Image contrast in panel A has been increased to accentuate areas of air trapping. FEV1 indicates forced expiratory volume in the first second of expiration.

Tables

References

CME


You need to register in order to view this quiz.

Multimedia

Some tools below are only available to our subscribers or users with an online account.

Web of Science® Times Cited: 61

Sign in

Create a free personal account to sign up for alerts, share articles, and more.

Purchase Options

• Buy this article
• Subscribe to the journal

Related Content

Customize your page view by dragging & repositioning the boxes below.

See Also...
Articles Related By Topic
Related Collections
PubMed Articles
Jobs
brightcove.createExperiences();