Eosinophilic Granuloma (Histiocytosis X) Treatment & Management

  • Author: Eleanor M Summerhill, MD, FACP, FCCP; Chief Editor: Zab Mosenifar, MD   more...
 
Updated: Jan 17, 2012
 

Medical Care

Smoking cessation is the most important medical intervention for pulmonary Langerhans cell histiocytosis X (PLCH). Smoking cessation often stabilizes the disease and sometimes leads to regression. It is also helpful in preventing bronchogenic carcinoma. Largely because of the rarity of PLCH, well-designed, prospective, randomized data regarding therapy are lacking. Treatment considerations are as follows:

  • The use of corticosteroids is controversial. Corticosteroids may be considered in patients with a persistence of clinically significant pulmonary or constitutional symptoms or those with documented progression of disease. Corticosteroid therapy is not indicated in patients with normal lung function. Recommendations for the use of corticosteroids are based largely on retrospective data and expert opinion.
  • Investigational therapies include interleukin-2 (IL-2) and anti–tumor necrosis factor-alpha (anti–TNF-alpha). Both agents have been reported to improve outcomes in pediatric disseminated histiocytosis. This finding may lead to the investigation of their use in adult PLCH.
  • Useful adjunctive therapies include the following:
    • Supplemental oxygen therapy for those with clinically significant hypoxemia (SaO2 < 89% or PaO2 < 55 mmHg) at rest or with exertion
    • Aggressive treatment for pulmonary infections with prompt initiation of antibiotic therapy
    • Bronchodilator therapy in the presence of an obstructive ventilatory defect
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Surgical Care

Lung transplantation is an option for select patients with advanced disease. Recurrence of pulmonary Langerhans cell histiocytosis X (PLCH) has been reported in the transplanted lung.[10]

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Consultations

Refer patients with suspected pulmonary Langerhans cell histiocytosis X (PLCH) to a pulmonary disease specialist.

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Activity

Exercise and pulmonary rehabilitation are encouraged in pulmonary Langerhans cell histiocytosis X (PLCH). These activities may improve the patient's functional status, even if they have no effect on disease progression.

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Contributor Information and Disclosures
Author

Eleanor M Summerhill, MD, FACP, FCCP  Associate Professor of Medicine, Division of Pulmonary and Critical Care Medicine, Warren Alpert Medical School of Brown University; Director, Internal Medicine Residency Program, Memorial Hospital of Rhode Island

Eleanor M Summerhill, MD, FACP, FCCP is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Thoracic Society, Association of Program Directors in Internal Medicine, and Rhode Island Medical Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael Peterson, MD  Chief of Medicine, Vice-Chair of Medicine, University of California, San Francisco, School of Medicine; Endowed Professor of Medicine, University of California, San Francisco-Fresno, School of Medicine

Michael Peterson, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, and American Thoracic Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Daniel R Ouellette, MD, FCCP  Associate Professor of Medicine, Wayne State University School of Medicine; Consulting Staff, Pulmonary Disease and Critical Care Medicine Service, Henry Ford Health System

Daniel R Ouellette, MD, FCCP is a member of the following medical societies: American College of Chest Physicians and American Thoracic Society

Disclosure: Nothing to disclose.

Timothy D Rice, MD  Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, St Louis University School of Medicine

Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians

Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD  Director, Division of Pulmonary and Critical Care Medicine, Director, Women's Guild Pulmonary Disease Institute, Professor and Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center, University of California, Los Angeles, David Geffen School of Medicine

Zab Mosenifar, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, and American Thoracic Society

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Robert S. Crausman, MD, MMS, to the development and writing of this article.

References

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Low-power photomicrograph of a lung-tissue specimen that demonstrates the classic stellate nodule of pulmonary histiocytosis X (hematoxylin-eosin stain).
 
 
 
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