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Histiocytosis: Symptoms and Treatment Information

  • Synopsis: Published: 2014-10-07 (Revised/Updated 2017-06-25) - Information regarding Histiocytosis, a group of rare disorders involving cells that usually have important roles as part of the immune system. For further information pertaining to this article contact: Thomas C. Weiss at Disabled World.
Histiocytosis

In medicine, histiocytosis refers to an excessive number of histiocytes, (tissue macrophages), and is typically used to refer to a group of rare diseases which share this as a characteristic. Occasionally and confusingly, the term "histiocytosis" is sometimes used to refer to individual diseases. Although nearly a century has passed since histiocytic disorders were recognized, their pathophysiology has started to be elucidated with the application of molecular analyses.

Langerhans Cell Histiocytosis Synonyms

  • LCH
  • histiocytosis X
  • Letterer-Siwe disease
  • eosinophilic granuloma
  • Abt-Letterer-Siwe disease
  • self-healing histiocytosis
  • Hashimoto-Pritzker syndrome
  • pure cutaneous histiocytosis
  • Hand-Schueller-Christian syndrome

Main Document

Quote: "The first sign of histiocytosis is often times a rash on the person's scalp, similar to cradle cap. The person may experience pain in a bone, discharge from their ear, a fever and loss of appetite."

Histiocytosis is also referred to as, "Langerhans Cell Histiocytosis (LCH)," or more formally as, "Histiocytosis X," and represents a group of rare disorders involving specific cells that usually have important roles as part of a person's immune system.

While the cause of LCH is still not known, it can frequently behave like cancer and because of this is treated by cancer specialists. 'Histiocytosis,' is a generic name for a group of syndromes characterized by an abnormal increase in the number of certain immune cells called, 'histiocytes,' which include macrophages, monocytes and dendritic cells.

A, 'histiocyte,' is an immune cell that is found in several parts of a person's body, especially in a person's:

  • Skin
  • Liver
  • Lungs
  • Spleen
  • Blood stream
  • Bone marrow
  • Lymph glands

In histiocytosis, the histiocytes move into a person's tissues where they are not usually found and cause damage to those tissues. These proliferating immune cells might form tumors, which can affect various parts of the person's body.

LCH is often times classified as a single system, when the disease affects only one part of the person's body, or multisystem when it affects more than one part of the person's body. In children, histiocytosis usually involves the bones and might consist of single or multiple sites. The child's skull is frequently affected. Children over the age of five usually have the single system disease, with just bone involvement. Young children, particularly infants, are more likely to experience the multisystem disease.

The majority of instances of histiocytosis affect children between the ages of one and fifteen years, although people of all ages may develop LCH. The incidence peaks among children between the ages of five and ten. Histiocytosis is thought to affect about one to two out of 200,000 people every year. The exact cause of histiocytosis remains unknown at this time. Recent studies; however, indicate that it is caused by the development and expansion of an abnormal Langerhans cell that subsequently leads to the accumulation of other cells of the immune system, resulting in collections or tumors in various areas of a person's body. Some forms are genetic.

Symptoms of Histiocytosis

The first sign of histiocytosis is often times a rash on the person's scalp, similar to cradle cap. The person may experience pain in a bone, discharge from their ear, a fever and loss of appetite. At times the person's stomach becomes swollen and painful. On occasion, an area of the person's brain known as the pituitary gland is affected and may lead to the person passing large amounts of urine and being very thirsty. Additional potential signs and symptoms include the following:

  • Limping
  • Seizures
  • Jaundice
  • Vomiting
  • Dizziness
  • Headache
  • Weight loss
  • Short stature
  • Delayed puberty
  • Generalized rash
  • Protruding eyeballs
  • Mental deterioration

The tumors produce a, 'punched-out,' appearance on X-rays of the person's bones. At times, children experience spontaneous fractures because of the bone lesions. An affected person often times experiences systemic involvement too, which might affect their entire body and cause symptoms which include the following:

  • Rashes
  • Anemia
  • Lung issues
  • Gum infiltration
  • Hormonal problems
  • Lymph gland swelling
  • Enlargement of the spleen and liver

Diagnosing Histiocytosis

Diagnostic testing for histiocytosis includes some different things such as a biopsy, in which a small sample of the person's skin or bone is taken and examined under a microscope for abnormal cells. Routine and sometimes specialized X-rays and scans of the person's bones, skull, and lungs may be pursued. Blood testing may also be performed. The tests will help a doctor to determine if the disease is the single system or multisystem type. An X-ray of the person's entire skeletal system might be done to determine how extensive the disease is and whether or not the systemic involvement is indicated.

Treating Histiocytosis and Research

Depending on the extent of the disease, LCH is often times treated with chemotherapy and steroids to suppress the function of the immune system and the production of histiocytes. The length of treatment varies from person to person. A number of people are eligible for international as well as local institutional trials.

Radiation therapy, treatment with targeted X-rays, or limited surgery might also be used to treat bone lesions in some situations. The majority of people who develop histiocytosis completely recover. At times the disease may recur, so the person will have regularly scheduled follow-up visits in an outpatient clinic as a precaution.

New ideas are being tested to determine the causes of LCH, as well as why some people respond better to treatment than others do. New types of therapies are being developed, to include new types of medications, as are approaches that direct antibodies or small molecules to the abnormal Langerhans cell while sparing regular tissues.

Awareness: Histiocytosis Awareness Information

Navy blue awareness ribbonThe awareness ribbon color for Histiocytosis is dark or navy blue.

The month of September is Histiocytosis awareness month, this is the time the Histio Community joins together to raise awareness for histiocytosis.

Facts: Histiocytosis

There are three major classes of histiocytosis:

  • Langerhans cell histiocytosis, which is also called histiocytosis X
  • Malignant histiocytosis syndrome (now known as T-cell lymphoma)
  • Non-Langerhans cell histiocytosis (also known as hemophagocytic syndrome)

LCH in infants may affect the skin only. In some cases, skin-only LCH may get worse over weeks or months and become a form called high-risk multisystem LCH.

Pulmonary histiocytosis X is a specific type of histiocytosis X that involves swelling of the small airways and small blood vessels in the lungs.

Histiocytosis X often affects the whole body. A disease that affects the whole body is called a "systemic" disorder.

Statistics: Histiocytosis

  • Histiocytosis Association of America (HAA) states that most of the people diagnosed with histiocytosis are children under the age of 10, although the disease can afflict adults.
  • The University of California, San Francisco, states that the disease usually occurs from birth to age 15.
  • 1 in 200,000 children in the United States are born with histiocytosis each year.




Information from our Autoimmune Diseases: Types, Facts & Information section - (Full List).

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