Interval History • The patient was admitted to respiratory isolation to rule out active infection with Mycobacterium Tuberculosis • Acid-fast bacilli culture and smear were negative • The patient improved on empiric NSAIDs and was discharged home • The patient returned to her baseline exercise tolerance and was asymptomatic within 1 week
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Interval History The patient was admitted to respiratory isolation to rule out active infection with Mycobacterium Tuberculosis Acid-fast bacilli culture.
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Interval History
• The patient was admitted to respiratory isolation to rule out active infection with Mycobacterium Tuberculosis
• Acid-fast bacilli culture and smear were negative
• The patient improved on empiric NSAIDs and was discharged home
• The patient returned to her baseline exercise tolerance and was asymptomatic within 1 week
Interval History
• Repeat CT imaging showed resolution of effusions, but persistent nodules, concerning for metastatic disease of unknown primary
• PET Scan and Abdominal CT scans did not show evidence of extrapulmonary malignancy
• The patient returned two months later for a thorascopy with lung wedge resection of a characteristic nodule
4x
10x
20x
40x
CD1a
S100
PULMONARY LANGERHANS’- CELL HYSTIOCYTOSIS
Final Pathological Diagnosis
PLCH: Introduction
• Histiocytosis encompasses a group of diverse disorders with the common primary event of the accumulation and infiltration of monocytes, macrophages, and dendritic cells in the affected tissues
• Langerhans Cells are a dendritic cell subtype and part of the monocyte-macrophage lineage derived from bone marrow involved in antigen presentation in the tracheobronchial tree
Classification of Histiocytosis
• Single-organ involvemento Lung (>85% of lung involvement occurs in isolation)o Boneo Skino Pituitaryo Lymph Nodeso Thyroid, Liver, Spleen, Brain
• Multisystem Diseaseo Multiorgan disease with lung involvemento Multiorgan disease without lung involvemento Multiorgan histiocytic disorder
o A rare systemic aggressive disease seen in adults
• Hand-Schüller-Christian diseaseo Triad of exopthalmos, central diabetes
insipidus, and bone lesions
Langerhans Cells
• Discovered by Paul Langerhans in 1868
• The hallmark ultrastructural feature called the Birbeck Granule discovered in 1961
• The CD1a cell surface antigen distinguises LC from other histiocytes
Epidemiology
• Precise incidence and prevalence is hard to define in this diseaseo 1200 new cases per yearo 0.5-5.4 cases / million
• 5% of lung-biopsy specimens in patients with ILD result in PLCH
• No known genetic susceptibility• Mainly in caucasians. Male to female
ratio is changing over the decades…• >90% of PLCH patients are smokers
Vassallo R et al. N Engl J Med 2000;342:1969-1978
Proposed Pathogenesis of PLCH
Reactive vs. Neoplastic?
• Spontaneous remission
• Abscence of chromosomal abnormalities
• Overall good prognosis in majority of cases
• Monoclonal proliferation in extrapulmonary tissue
• Infiltration of aberrent cells into normal tissue
• Response to chemotherapy and possible fatal outcome in more severe cases
Histopathological Features
• Proliferation of Lagerhans Cells along the small airways serves as the nidus of cellular/fibrotic nodules from 5mm to 1.5 in size. Eosinophils may be present
• In severe disease, nodules may interconnect and cavitate to produce distinctive honeycomb-like structures
• Given that most patients are smokers, concominant COPD and ILD 2/2 respiratory bronchiolitis is often present
o 2-chlorodeoxyadenosine• Immune modulation: Etanercept• Pleurodesis of pneumothoraces• Serial TTE and PFTs to monitor
progression
Prognosis
• For a majority of patients, the disease regresses with smoking cessation
• It is not known to predict those who tend to progress, although age, prolonged constitutional symptoms, extrapulmonary involvement, abnormal PFTs are markers of poor outcome
Back to our case…
• This patient has baseline respiratory insufficiency 2/2 PLCH and COPD, but presented with an acute respiratory illness not characteristic of these diagnoses
• She endorsed chills, dyspnea, and chest pain. There was radiographic evidence of pleuropericarditis which symptomatically and radiographically improved within 1-2 weeks on NSAIDs