Histiocytosis: Symptoms, Treatment, Information

Introduction

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.

The U.S. Social Security Administration (SSA) has included Histiocytosis as a Compassionate Allowance to expedite a disability claim.

Main Digest

While the cause of LCH is still unknown, it can frequently behave like cancer and is treated by cancer specialists. 'Histiocytosis' is a generic name for a group of syndromes characterized by an abnormal increase in 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 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 five usually have a single-system disease with bone involvement. Young children, particularly infants, are more likely to experience 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 affects about one to two out of 200,000 people every year. The exact cause of histiocytosis remains unknown at this time. Recent studies 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 a rash on the person's scalp, similar to a cradle cap. The person may experience pain in a bone, discharge from the 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 experiences systemic involvement too, which might affect their entire body and cause symptoms that 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 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 determine if the disease is a 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 systemic involvement is indicated.

Treating Histiocytosis and Research

Depending on the extent of the disease, LCH is often 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. Several 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 and why some people respond better to treatment than others. New therapies are being developed, including new medications, as are approaches that direct antibodies or small molecules to the abnormal Langerhans cell while sparing normal tissues.

Histiocytosis Facts and Information

In medicine, histiocytosis refers to an excessive number of histiocytes (tissue macrophages) and is typically used to refer to a group of rare diseases that share this 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

Histiocytosis Association of America (HAA) states that most people diagnosed with histiocytosis are children under 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 is born with histiocytosis yearly. 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 a 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 involving swelling of the small airways and blood vessels in the lungs.

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

Author Credentials:

Thomas C. Weiss is a researcher and editor for Disabled World. Thomas attended college and university courses earning a Masters, Bachelors and two Associate degrees, as well as pursing Disability Studies. As a Nursing Assistant Thomas has assisted people from a variety of racial, religious, gender, class, and age groups by providing care for people with all forms of disabilities from Multiple Sclerosis to Parkinson's; para and quadriplegia to Spina Bifida. Explore Thomas' complete biography for comprehensive insights into his background, expertise, and accomplishments.

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Cite This Page (APA): Weiss, T. C. (2014, October 7). Histiocytosis: Symptoms, Treatment, Information. Disabled World. Retrieved May 20, 2024 from www.disabled-world.com/health/autoimmunediseases/histiocytosis.php

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