Table of Contents
- Overview
- Symptoms
- Treatment
- Prevention
- Images
Histiocytosis X; Langerhans cell histiocytosis; Eosinophilic granuloma; Pulmonary histiocytosis X; Nonlipid reticuloendotheliosis; Pulmonary Langerhans cell granulomatosis; Hand-Schuller-Christian disease; Letterer-Siwe disease
Symptoms
Histiocytosis X often affects the whole body. A disease that affects the whole body is called a systemic disorder.
Symptoms can vary between children and adults, although there can be some overlap. Tumors in weight-bearing bones, such as the legs or spine, may cause the bones to fracture without apparent reason.
Symptoms in children may include:
- Abdominal pain
- Bone pain (possibly)
- Delayed puberty
- Dizziness
- Ear drainage that continues long-term
- Eyes that appear to stick out (protrude) more and more
- Irritability
Failure to thrive - Fever
- Frequent urination
- Headache
- Jaundice
- Limping
- Mental deterioration
- Rash (
petechiae orpurpura ) -
Seborrheic dermatitis of the scalp - Seizures
- Short stature
- Swollen lymph glands
- Thirst
- Vomiting
- Weight loss
Note: Children over 5 years old often have only bone involvement.
Symptoms in adults may include:
- Bone pain
Chest pain Cough Fever - General discomfort, uneasiness, or ill feeling (
malaise ) - Increased amount of urine
- Rash
Shortness of breath - Thirst and increased drinking of fluids
Weight loss
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.
Tests in children may also include:
- Biopsy of skin to check for the presence of Langerhans cells
-
Bone marrow biopsy to check for the presence of Langerhans cells - Complete blood count (
CBC ) -
X-rays of all the bones in the body (skeletal survey) to find out how many bones are affected
Tests in adults may include:
-
Bronchoscopy withbiopsy Chest x-ray Pulmonary function tests
Histiocytosis X is sometimes associated with cancer. CT scans and biopsy should be done to rule out possible cancer.
Previous Section
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)
