Friday, February, 10, 2012

Histiocytosis

Table of Contents

Alternative Names

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


Treatment

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 outlook. Such medications may include:

  • Cyclophosphamide
  • Etoposide
  • Methotrexate
  • Vinblastine

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

Other treatments may include:

  • Antibiotics to fight infections
  • Breathing support (with a breathing machine)
  • Hormone replacement therapy
  • Physical therapy
  • Special shampoos for scalp problems
  • Supportive care to relieve symptoms

Support Groups

Histiocytosis Association of America -- www.histio.org


Expectations (prognosis)

Histiocytosis X affects many organs and can lead to death.

About half of those with pulmonary histiocytosis see improvement, while others eventually have permanent loss of lung function.

In very young patients, the outlook depends on the specific histiocytosis and severity of the disease. Some children can live a normal life with minimal disease involvement, while others may have a poor outcome. Young children, especially infants, are more likely to have body-wide symptoms that lead to death.


Complications

Complications may include:

  • Diffuse interstitial pulmonary fibrosis
  • Spontaneous pneumothorax

Children may also develop:

  • Anemia caused by spreading of the tumors to the bone marrow
  • Diabetes insipidus
  • Lung problems that lead to lung failure
  • Problems with the pituitary gland that lead to growth failure

Calling your health care provider

Call your health care provider if you or your child have symptoms of this disorder. Go to the emergency room if shortness of breath or chest pain develop.


Review Date: 04/30/2010
Reviewed By: Todd Gersten, M.D., Hematology/Oncology, Palm Beach Cancer Institute, West Palm Beach, FL. Review provided by VeriMed Healthcare Network.Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org)