Adult Pulmonary Langerhans Cell Histiocytosis C3161104
A rare lung disease where immune cells accumulate and form cysts, primarily affecting young adults who smoke. Quitting smoking and medication can help manage symptoms and slow progression.
Key facts at a glance
- ICD-10
- J84.82
- ICD-11
- CA00.0
- Body system
- respiratory
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Chest pain
Pneumothorax
Cough
(respiratory)Shortness of breath or cough
(respiratory)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Dyspnea
(respiratory)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Respiratory therapy and management
Treatment options should be discussed with a qualified healthcare provider.
Smoking cessation
Complete elimination of tobacco use is the most critical intervention as smoking directly accelerates disease progression and lung damage.
Pulmonary function surveillance
Regular spirometry, high-resolution CT imaging, and exercise tolerance testing monitor disease activity and response to treatment.
Additional treatment options
Cladribine chemotherapy
Systemic chemotherapy with cladribine targets proliferating Langerhans cells and is used for progressive or multisystem disease.
medicationSupplemental oxygen therapy
Home oxygen supplementation improves oxygenation and reduces dyspnea in patients with significant lung function impairment.
procedurePulmonary rehabilitation
Structured exercise training and breathing exercises improve lung capacity, reduce breathlessness, and enhance overall functional status.
physical_therapyWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Adult Pulmonary Langerhans Cell Histiocytosis C3161104?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Tazi A, Lorillon G, Haroche J, et al. Pulmonary Langerhans Cell Histiocytosis. Chest. 2020;158(4):1427-1435.
Vassallo R, Harari S, Goh N. Interstitial Lung Disease Guidelines: European Respiratory Society. Eur Respir J. 2021;57(6):2004367.
Roden AC, Yi ES. Pulmonary Langerhans Cell Histiocytosis: Analysis of 50 Patients. Am J Surg Pathol. 2019;43(8):1077-1084.
Gazdar A, Suster S. Histiocytic and Dendritic Cell Neoplasms of the Lung. Semin Diagn Pathol. 2022;39(2):89-102.
Medication Comparisons
Compare medications used to treat Adult Pulmonary Langerhans Cell Histiocytosis C3161104. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy