Histiocytosis

  • 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.