Histiocytosis

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Eosinophilic granuloma - X-ray of the skull

Respiratory system

Eosinophilic granuloma; Pulmonary histiocytosis X; Nonlipid reticuloendotheliosis; Pulmonary Langerhans cell granulomatosis; Hand-Schuller-Christian disease; Letterer-Siwe disease; Langerhans cell histiocytosis

This disorder is treated with corticosteroids, which suppress immune function (including the dangerous cells). Smoking may worsen the response to treatment and should be stopped.

Children may be given other medications depending on their estimated prognosis, including:

• Methotrexate
• Vinblastine
• Cyclophosphamide
• Etoposide

Radiation therapy or limited surgery may also be used to treat bone lesions.

Patients are also treated with supportive care to reduce symptoms and treat infections that may occur when the immune system is compromised. Treatment may include antibiotics, breathing assistance with a respirator, physical therapy, selenium-based shampoo for scalp problems, and hormone replacement to deal with hormonal dysfunction.

For additional information, patients may wish to contact the Histiocytosis Association of America at 800-548-2758.

In pulmonary histiocytosis, the disease stabilizes or improves in about one-half of the affected people and the other half progress to a permanent loss of lung function.

In the pediatric versions, the outlook depends on the actual disease category and severity of the disease. Some can be expected to have a normal life span with minimal disease involvement, and some can expect a poor outcome.

• Spontaneous pneumothorax
• Diffuse interstitial pulmonary fibrosis

Children may also develop:

• Pituitary dysfunction with growth failure
• Diabetes insipidus
• Bone marrow invasion by the tumors, with subsequent anemia
• Lung problems with eventual lung failure

Call your health care provider or go to the emergency room (depending on the extent and severity of symptoms) if shortness of breath, chest pain, or other symptoms of this disorder develop.

Review Date: 10/30/2006
Reviewed By: William Matsui, MD, Assistant Professor of Oncology, Division of Hematologic Malignancies, The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, Baltimore, MD. Review provided by VeriMed Healthcare Network.