Eosinophilic Granuloma (Histiocytosis X) Treatment & Management

  • Author: Eleanor M Summerhill, MD, FACP, FCCP; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
 
Updated: Jun 03, 2016
 

Approach Considerations

Inpatient admission is indicated in pulmonary Langerhans cell histiocytosis X (PLCH) patients only to manage complications related to the disease as listed below.

Patients with superimposed respiratory infections, such as pneumonia, may require inpatient treatment.

Patients with spontaneous pneumothorax may require chest-tube placement and subsequent in-patient care. The recurrence rate of secondary spontaneous pneumothorax in PLCH is high. Therefore, some experts recommend surgical intervention, such as mechanical pleurodesis, parietal pleurectomy, or talc insufflation, to prevent further occurrences after the initial episode.

Acute respiratory failure necessitating in-patient management may occur as the result of a superimposed respiratory infection or spontaneous pneumothorax. Respiratory failure may also occur as a manifestation of end-stage disease.

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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.

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 (SaO 2 < 89% or PaO 2 < 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.[11]

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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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Complications

Spontaneous pneumothorax is a common complication (10-20%) in pulmonary Langerhans cell histiocytosis X (PLCH).

PLCH is associated with an increased risk of malignancy, including Hodgkin and non-Hodgkin lymphoma, myeloproliferative disorders, and bronchogenic carcinoma.[12]

Pathologic fracture may occur at the site of bone lesions.

Diabetes insipidus occurs in 10-15% of patients and indicates disease in the central nervous system.

Pulmonary artery hypertension and cor pulmonale may develop as a result of hypoxemia and/or vascular disruption due to PLCH lesions.

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Prevention

Effective antismoking measures can prevent pulmonary Langerhans cell histiocytosis X (PLCH). See Medscape's Smoking Resource Center.

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Long-Term Monitoring

In the care of patients with pulmonary Langerhans cell histiocytosis X (PLCH) , important considerations include the patients' smoking history and current smoking status, the presence or absence of extrapulmonary disease and constitutional symptoms, and close monitoring for progression of pulmonary disease.

Pulmonary artery hypertension is a known complication of infiltrative lung diseases, and in PLCH the magnitude of pulmonary artery hypertension may be greater than expected for given the degree of hypoxemia or level of impairment on pulmonary function testing.

The increased risk of pulmonary malignancies must be considered.

Smoking cessation counseling and adjunctive pharmacologic therapy with bupropion and nicotine replacement are key components of long-term management.

Perform pulmonary function testing and radiographic studies every 3-6 months, as the patient's clinical condition warrants.

Assess arterial oxygen saturation both at rest and with activity.

Echocardiography should be considered in all patients with clinically significant dyspnea in order to screen for pulmonary artery hypertension.

  • If echocardiographic results suggest moderate-to-severe pulmonary artery hypertension, these findings should be further evaluated and confirmed with right-heart catheterization.
  • At the time of catheterization, the response to vasodilators may also be assessed.

Patients should be vaccinated annually for influenza and should also receive the pneumococcal vaccine.

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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, Rhode Island Medical Society, Association of Program Directors in Internal Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Daniel R Ouellette, MD, FCCP Associate Professor of Medicine, Wayne State University School of Medicine; Chair of the Clinical Competency Committee, Pulmonary and Critical Care Fellowship Program, Senior Staff and Attending Physician, Division of Pulmonary and Critical Care Medicine, Henry Ford Health System; Chair, Guideline Oversight Committee, American College of Chest Physicians

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

Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD, FACP, FCCP Geri and Richard Brawerman Chair in Pulmonary and Critical Care Medicine, Professor and Executive Vice Chairman, Department of Medicine, Medical Director, Women's Guild Lung Institute, Cedars Sinai Medical Center, University of California, Los Angeles, David Geffen School of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

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, American Thoracic Society

Disclosure: Nothing to disclose.

Acknowledgements

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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  8. Delobbe A, Durieu J, Duhamel A, Wallaert B. Determinants of survival in pulmonary Langerhans' cell granulomatosis (histiocytosis X). Groupe d'Etude en Pathologie Interstitielle de la Société de Pathologie Thoracique du Nord. Eur Respir J. 1996 Oct. 9(10):2002-6. [Medline].

  9. DiCaprio MR, Roberts TT. Diagnosis and Management of Langerhans Cell Histiocytosis. J Am Acad Orthop Surg. 2014 Oct. 22(10):643-652. [Medline].

  10. Stacher E, Beham-Schmid C, Terpe HJ, Simiantonaki N, Popper HH. Pulmonary histiocytic sarcoma mimicking pulmonary Langerhans cell histiocytosis in a young adult presenting with spontaneous pneumothorax: a potential diagnostic pitfall. Virchows Arch. 2009 Aug. 455(2):187-90. [Medline].

  11. Alalawi R, Whelan T, Bajwa RS, Hodges TN. Lung transplantation and interstitial lung disease. Curr Opin Pulm Med. 2005 Sep. 11(5):461-6. [Medline].

  12. Misaki H, Yamauchi T, Arai H, et al. Secondary malignant fibrous histiocytoma following refractory langerhans cell histiocytosis. J Clin Exp Hematop. 2009 May. 49(1):33-7. [Medline].

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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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