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Utility of bronchoscopy in pulmonary Langerhans cell histiocytosis
Misbah Baqir1, Robert Vassallo, Fabien Maldonado
1Divisions of *Pulmonary and Critical Care Medicine †Anatomic Pathology, Mayo Clinic, Rochester, MN.
Insights
Bronchoscopic lung biopsy is a useful initial diagnostic tool for Pulmonary Langerhans cell histiocytosis (PLCH), a rare smoking-related lung disease. This method aided diagnosis in 50% of patients, making it a valuable procedure for identifying PLCH.
Area of Science:
- Pulmonology
- Pathology
- Thoracic Surgery
Background:
- Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon interstitial lung disease, often associated with smoking in adults.
- Limited data exist on the effectiveness of bronchoscopic lung biopsy for diagnosing PLCH.
Purpose of the Study:
- To evaluate the diagnostic utility of bronchoscopic lung biopsy in patients with Pulmonary Langerhans cell histiocytosis.
- To determine the role of bronchoscopic biopsy as an initial diagnostic procedure for PLCH.
Main Methods:
- A retrospective review of 38 patients diagnosed with PLCH between 1997 and 2012 who underwent bronchoscopy with lung biopsy.
- Data extraction included demographics, clinical features, imaging, and biopsy results.
- Diagnostic criteria for PLCH involved histopathology, CD1a staining in bronchoalveolar lavage (BAL), or extrapulmonary biopsy.
Main Results:
- Bronchoscopic biopsy provided diagnostic specimens in 19 out of 38 patients (50%).
- CD1a immunostaining of BAL cells, performed in 8 patients, identified PLCH in 3 additional cases (8%).
- The majority of patients (84%) were active smokers at diagnosis.
Conclusions:
- Bronchoscopic lung biopsy is a valuable tool for diagnosing Pulmonary Langerhans cell histiocytosis.
- It should be considered the primary method for obtaining diagnostic tissue samples in suspected PLCH cases.
Background:
Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon form of interstitial lung disease and is usually smoking-related when seen in adults. There are relatively little data regarding the utility of bronchoscopic lung biopsy for this disorder.
Methods:
A computer-assisted search was carried out to identify patients with PLCH seen at Mayo Clinic Rochester, MN from 1997 to 2012 and who underwent bronchoscopy with lung biopsy. Approval was obtained from the Mayo Foundation Institutional Review Board before beginning the study. Medical records of these patients were reviewed to extract data with regard to demographic and clinical features, imaging studies, and biopsy results.
Results:
Thirty-eight patients with PLCH underwent diagnostic bronchoscopy with biopsies. Their median age was 39.5 years (range, 21 to 66 y) and included 24 women. Thirty-two patients (84%) were current smokers at the time of the diagnosis, 5 were ex-smokers (13%), and 1 was a never-smoker (3%). The diagnosis of PLCH required the presence of typical histopathologic features on surgical or bronchoscopic lung biopsy, >5% CD1a-positive cells in the bronchoalveolar lavage (BAL), and/or biopsy of an extrapulmonary site in the presence of clinical and chest computed tomographic findings compatible with the diagnosis. Bronchoscopic biopsy yielded diagnostic specimens that allowed the diagnosis of PLCH in 19 patients (50%). CD1a immunostaining of BAL cells had been performed in 8 patients and demonstrated ≥ 5% CD1a-positive BAL cells in 3 additional patients (8%).
Conclusions:
We conclude that bronchoscopic lung biopsy is useful in the diagnosis of PLCH and should be the initial method of obtaining diagnostic specimens.
