Saturday, February, 11, 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


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 or purpura)
  • 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 with biopsy
  • 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.


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)