Background:
Bronchiolitis obliterans is a form of chronic graft-versus-host disease (GVHD) that sometimes
develops after stem cell transplantation (SCT) or bone marrow transplantation (BMT).
In bronchiolitis obliterans, immune cells that normally fight infections attack the lungs of
the transplant recipient, causing destruction of lung tissue and fibrosis (scarring). When
fibrosis develops, the lungs cannot work properly.
Montelukast (Singulair) is a drug that has been used for many years to treat asthma. Its use
as a treatment for bronchiolitis obliterans is experimental.
Objectives:
To see if montelukast improves or stabilizes lung function in patients who develop
bronchiolitis obliterans after BMT or SCT.
To assess the safety of montelukast in patients with bronchiolitis obliterans after BMT or
SCT
To see if montelukast affects the cells that damage the lungs.
To see if montelukast improves other forms of chronic GVHD, quality of life, and overall
survival in patients with bronchiolitis obliterans after BMT or SCT.
Eligibility:
Patients 6 years of age and older with bronchiolitis obliterans following stem cell
transplantation.
Design:
Patients take one montelukast tablet daily for 6 months and undergo the following procedures
during this period:
- Lung function tests. The patient breathes into a machine that measures the amount of air
that goes into and out of the lungs. This test is done once a month for 3 months, then
at 6 months, 12 months and 24 months.
- Medical history and physical examination at the study site about every 3 months for the
first year of the study and then at 12 months and 24 months. Patients also have physical
examinations monthly for the first 6 months at their primary doctors office. Tests may
include blood and urine tests, chest computed tomography (CT) scans, echocardiogram
(heart ultrasound), 2- and 6-minute walk tests, and quality-of-life questionnaires.
- Bronchoalveolar lavage in patients 18 years of age and older. The subject s mouth, nose
and airways are numbed with lidocaine. Some patients may need sedation or anesthesia for
the procedure. A tube (bronchoscope) is then passed through the nose into the airway,
and a small amount of fluid is put into the lung. The fluid is then removed and tested
for infections or other lung problems.
- Apheresis to collect white blood cells. Whole blood is collected through a tube inserted
into a vein in the arm. The white cells are extracted in a cell separator machine, and
the rest of the blood is returned to the body through a tube placed in a vein in the
other arm. The cells are used to study GVHD and bronchiolitis obliterans.
- Patients who wish to continue montelukast therapy after 6 months may do so under the
care of their primary doctor, if both agree to the continuation....