Histiocytosis

Alternative names:

eosinophilic granuloma; Hand-Schuller-Christian disease; histiocytosis X; Langerhans cell histiocytoses; Letterer-Siwe disease; nonlipid reticuloendotheliosis; pulmonary histiocytosis X; pulmonary langerhans cell granulomatosis

Treatment:

This disorder is treated with corticosteroids. Stopping smoking may improve the response to treatment.

Children may be given other medications, including:

• prednisone
• methotrexate
• vinblastine
• cyclophosphamide
• etoposide

Radiation therapy may also be used to treat bone lesions.

Expectations (prognosis):

The disease stabilized or improves in about one-half of the affected people and the other half progress to a permanent loss of lung function.

Complications:

• spontaneous pneumothorax
• diffuse interstitial pulmonary fibrosis

Children may also develop:

• pituitary dysfunction with growth failure
• diabetes insipidus
• bone marrow invasion with subsequent anemia
• lung problems with eventually pulmonary failure

Calling your health care provider:

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.