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
Definition of Histiocytosis
Histiocytosis is a general name for a group of syndromes that involve an abnormal increase in the number of immune cells called histiocytes.
Causes, incidence, and risk factors
Histiocytosis X has typically been thought of as a cancer-like condition. More recently researchers have begun to suspect that it is actually an autoimmune phenomenon, in which immune cells mistakenly attack the body, rather than fight infections. Extra immune cells may form tumors, which can affect various parts of the body including the bones, skull, and other areas.
Symptoms
Histiocytosis X often affects the whole body. A disease that affects the whole body is called a systemic disorder.
Signs and tests
The tumors produce a “punched-out” look on a bone x-ray. Specific tests vary depending on the age of the patient.
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.
Expectations (prognosis)
Histiocytosis X affects many organs and can lead to death.
Review
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. – 4/30/2010
