Histiocytosis is a general name for a group of syndromes that involve an abnormal increase in the number of immune cells called histiocytes.
There are three major classes of histiocytoses:
This article focuses only on Langerhans cell histiocytosis (histiocytosis X).
Alternative NamesHistiocytosis X; Langerhans cell histiocytosis; Eosinophilic granuloma; Pulmonary histiocytosis X; Nonlipid reticuloendotheliosis; Pulmonary Langerhans cell granulomatosis; Hand-Schuller-Christian disease; Letterer-Siwe disease
Causes, incidence, and risk factorsHistiocytosis X has typically been thought of as a cancer-like condition. More recently researchers have begun to suspect that it is actually an autoimmune phenomenon, in which immune cells mistakenly attack the body, rather than fight infections. Extra immune cells may form tumors, which can affect various parts of the body including the bones, skull, and other areas.
Some forms of the disorder are genetic.
Histiocytosis X is thought to affect roughly 1 in 200,000 people each year. It is most often seen in children ages 1 to 15. The rate peaks among children ages 5 to10.
Pulmonary histiocytosis X is a specific type of this disorder that involves swelling of the small airways (bronchioles) and small blood vessels in the lungs. It is most common in adults. The inflammation leads to lung stiffening and damage. The cause is unknown. It most often affects those ages 30 to 40, usually cigarette smokers.
SymptomsHistiocytosis 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:
Note: Children over 5 years old often have only bone involvement.
Symptoms in adults may include:
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:
Tests in adults may include:
Histiocytosis X is sometimes associated with cancer. CT scans and biopsy should be done to rule out possible cancer.
TreatmentThis disorder is treated with corticosteroids, which suppress immune function (including the dangerous cells). Smoking may worsen the response to treatment and should be stopped.
Children may be given other medications depending on their estimated outlook. Such medications may include:
Radiation therapy or surgery may also be used to treat bone lesions.
Other treatments may include:
Histiocytosis Association of America -- www.histio.org
Expectations (prognosis)Histiocytosis X affects many organs and can lead to death.
About half of those with pulmonary histiocytosis see improvement, while others eventually have permanent loss of lung function.
In very young patients, the outlook depends on the specific histiocytosis and severity of the disease. Some children can live a normal life with minimal disease involvement, while others may have a poor outcome. Young children, especially infants, are more likely to have body-wide symptoms that lead to death.
ComplicationsComplications may include:
Children may also develop:
Call your health care provider if you or your child have symptoms of this disorder. Go to the emergency room if shortness of breath or chest pain develop.
PreventionAvoid smoking. Quitting smoking can improve the outcome in people with histiocytosis that affects the lungs.
There is no known prevention for the childhood forms of the disease.
ReferencesLadisch S. Histiocytosis syndromes of childhood. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 507.
Raghu G. Interstitial lung disease. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 92.
Lipton JM, Arceci RJ. Histiocytic Disorders. In: Hoffman R, Benz EJ, Shattil SS, et al, eds. Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2008:chap 52.
Histiocytosis is a general name for a group of syndromes that involve an abnormal increase in the number of immune cells called histiocytes.
There are three major classes of histiocytoses:
This article focuses only on Langerhans cell histiocytosis (histiocytosis X).
Alternative NamesHistiocytosis X; Langerhans cell histiocytosis; Eosinophilic granuloma; Pulmonary histiocytosis X; Nonlipid reticuloendotheliosis; Pulmonary Langerhans cell granulomatosis; Hand-Schuller-Christian disease; Letterer-Siwe disease
Causes, incidence, and risk factorsHistiocytosis X has typically been thought of as a cancer-like condition. More recently researchers have begun to suspect that it is actually an autoimmune phenomenon, in which immune cells mistakenly attack the body, rather than fight infections. Extra immune cells may form tumors, which can affect various parts of the body including the bones, skull, and other areas.
Some forms of the disorder are genetic.
Histiocytosis X is thought to affect roughly 1 in 200,000 people each year. It is most often seen in children ages 1 to 15. The rate peaks among children ages 5 to10.
Pulmonary histiocytosis X is a specific type of this disorder that involves swelling of the small airways (bronchioles) and small blood vessels in the lungs. It is most common in adults. The inflammation leads to lung stiffening and damage. The cause is unknown. It most often affects those ages 30 to 40, usually cigarette smokers.
SymptomsHistiocytosis 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:
Note: Children over 5 years old often have only bone involvement.
Symptoms in adults may include:
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:
Tests in adults may include:
Histiocytosis X is sometimes associated with cancer. CT scans and biopsy should be done to rule out possible cancer.
TreatmentThis disorder is treated with corticosteroids, which suppress immune function (including the dangerous cells). Smoking may worsen the response to treatment and should be stopped.
Children may be given other medications depending on their estimated outlook. Such medications may include:
Radiation therapy or surgery may also be used to treat bone lesions.
Other treatments may include:
Histiocytosis Association -- www.histio.org
Expectations (prognosis)Histiocytosis X affects many organs and can lead to death.
About half of those with pulmonary histiocytosis see improvement, while others eventually have permanent loss of lung function.
In very young patients, the outlook depends on the specific histiocytosis and severity of the disease. Some children can live a normal life with minimal disease involvement, while others may have a poor outcome. Young children, especially infants, are more likely to have body-wide symptoms that lead to death.
ComplicationsComplications may include:
Children may also develop:
Call your health care provider if you or your child have symptoms of this disorder. Go to the emergency room if shortness of breath or chest pain develop.
PreventionAvoid smoking. Quitting smoking can improve the outcome in people with histiocytosis that affects the lungs.
There is no known prevention for the childhood forms of the disease.
ReferencesLadisch S. Histiocytosis syndromes of childhood. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 507.
Raghu G. Interstitial lung disease. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 92.
Lipton JM, Arceci RJ. Histiocytic Disorders. In: Hoffman R, Benz EJ, Shattil SS, et al, eds. Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2008:chap 52.
Reviewed ByReview Date: 04/30/2010
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.
Xanthogranuloma is a form of histiocytosis, an excess of histiocytes, with yellow lesions.
Histiocytosis X is a generic term that refers to an increase in the number of histiocytes, a type of white blood cell, that act as scavengers to remove foreign material from the blood and tissues.
eosinophilic granuloma, Hand-Schuller-Christian disease and Letterer-Siwe disease.
substances called cytokines and prostaglandins, which are normally produced by histiocytes and act as messengers between cells
Lymph nodes are small glands that function to filter the lymph (excess plasma that circulates around tissues in capillary beds); they are often the first place where infections and metastatic cancers are found. The sinuses of a lymph node are small open pockets between the lymphocytes where fluid can accumulate. Histiocytosis is an abnormal accumulation of histiocytes (also called macrophages). Therefore, lymph node sinus histiocytosis is an abnormal accumulation of macrophages within the open spaces of a lymph node. There are many potential causes of this, ranging from chronic infection to cancer. Your doctor will need to explain what the most likely cause is as well as treatment options and likely outcomes.
Epidermal (skin) Langerhans cells (a form of dendritic cell) accumulate with other immune cells in various parts of the body and cause damage by the release of chemicals.
the younger the child at diagnosis and the more organs involved, the poorer the outlook. If the condition resolves, there could still be long-term complications because of the damage done
symptoms may include painful lumps in the skull and limbs as well as rashes on the skin. General symptoms may include: poor appetite, failure to gain weight, recurrent fever, and irritability.
a biopsy, that is, taking a tissue sample under anesthesia from a site in the patient thought to be involved. Blood and urine tests, chest and other x rays, magnetic resonance imaging (MRI)
It's not that they lose the white blood cells, it's that the white blood cells are actually infected with AIDS. If they got the disease where they made too much, then more white blood cells would just get infected. The overall effect would be the same. The white blood cells can't kill HIV/AIDS because the virus is changing so much that your body can't really recognize it. Besides, histiocytosis (the disease where your body makes an excess of white blood cells) would probably just complicate the situation.
DefinitionA histiocyte is a type of immune cell that eats foreign substances in an effort to protect the body from infection.Alternative NamesMacrophageInformationHistiocytes do not travel through the blood. Instead, they remain in one part of the body.Histiocytes are found in many organs and tissues, including the following:BrainBreast tissueLiverLymph nodesPlacentaSpleenTonsilsAn abnormal number of histiocytes leads to a disease called histiocytosis X.ReferencesGoronzy JJ, Weyand Cm. The innate and adaptive immune systems. In: Goldman L, Ausiello D, eds. Cecil Medicine. Philadelphia, Pa: Saunders Elsevier;2007:chap 42.
Hello, A few of the diseases that diminish Lung capacity are: * Asthma. * Chronic Bronchitis. * COPD (Chronic Obstructive Pulmonary Disease). * Pneumonia that causes scarring of the Lungs in rare & severe cases can diminish Lung capacity. Please note this is advice only & should not be used in place of a medical experts.