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Encyclopedia > Langerhans cell histiocytosis
Langerhans cell histiocytosis
Classification & external resources
ICD-10 D76.0
ICD-9 202.5, 277.89
ICD-O: 9752/3
OMIM 604856
DiseasesDB 5906
eMedicine derm/216 

Langerhans Cell Histiocytosis (LCH) is a very rare disorder that each year is diagnosed in approximately 1 in 200,000 children under the age of 10, and even more rarely, in about 1 in 560,000 adults. While it often can be treated, it unfortunately is often debilitating and even fatal if diagnosed before age 2. Because it is so rare, not much is known about the disease, however, recent research and better communication about the disease has improved its recognition in the medical community.

Contents

History

Because the disease has a variety of different symptoms, depending upon which organs are affected, it used to be known by several different names including:

  • Histiocytosis-X;
  • Eosinophilic Granuloma when it affected a single body part;
  • Hand-Schuller-Christian Syndrome when referring to lesions of the bones and pituitary gland and protuberance of the eyes; and
  • Letterer-Siwe disease when referring to the severest form of the disease found in infants, involving lesions in the liver, bone marrow, spleen and skin.

Grays Anatomy illustration of a human femur. ... The pituitary gland, or hypophysis, is an endocrine gland about the size of a pea that sits in a small, bony cavity (pituitary fossa) covered by a membrane. ... This article refers to the sight organ. ... The liver is an organ in some animals, including vertebrates (and therefore humans). ... Grays Anatomy illustration of cells in bone marrow. ... The spleen is an organ of the upper abdomen, where it functions in the destruction of old red blood cells and holding a reservoir of blood. ... It has been suggested that this article or section be merged with Epidermis (skin). ...

Symptoms

While sometimes there may be generalised signs of illness such as loss of appetite, fever, and anaemia, investigation is usually prompted by one or more of the following symptoms: The appetite is the desire to eat food, felt as hunger. ... An analogue medical thermometer showing the temperature of 38. ... This article discusses the medical condition. ...

The symptom underlying all of these signs are histiocytic lesions on skin, bones, lung, liver, spleen, gums, ears, eyes, and/or the central nervous system. A typical rash A rash is a change in the skin which affects its appearance or texture. ... An infant with Cradle Cap Close up image of Cradle Cap Cradle Cap (Infantile seborrhoeic dermatitis, or crusta lactea, milk crust, honeycomb disease; croûte de lait, eczéma infantile, dermatite atopique infantile (French); ne shqip njihet si - rrjebull ose rrjebulla (Albanian); crosta lattea (Italian); and σμηγματόρροια του τριχωτού της κεφαλής των νεογνών (Greek)) is a patchy... Look up Discharge in Wiktionary, the free dictionary. ... Diabetes insipidus (DI) is a disease characterized by excretion of large amounts of severely diluted urine, which cannot be reduced when fluid intake is reduced. ... The pituitary gland, or hypophysis, is an endocrine gland about the size of a pea that sits in a small, bony cavity (pituitary fossa) covered by a membrane. ... Exophthalmos (or proptosis) is a bulging of the eye anteriorly out of the orbit. ...


Diagnosis

Initial diagnosis is made by tissue biopsy. Followup investigation is required to determine the extent of the lesions, and may include X-ray imaging to find lesions in the lungs and bone, blood tests to determine if there is an effect on liver function and red blood cell production and a bone marrow biopsy is also used to determine if there are bone marrow lesions. A biopsy (in Greek: bios = life and opsy = look/appearance) is a medical test involving the removal of cells or tissues for examination. ... In the NATO phonetic alphabet, X-ray represents the letter X. An X-ray picture (radiograph) taken by Röntgen An X-ray is a form of electromagnetic radiation with a wavelength approximately in the range of 5 pm to 10 nanometers (corresponding to frequencies in the range 30 PHz... Medical imaging designates the ensemble of techniques and processes used to create images of the human body (or parts thereof) for clinical purposes (medical procedures seeking to reveal, diagnose or examine disease) or medical science (including the study of normal anatomy and function). ... Blood tests are laboratory tests done on blood to gain an appreciation of disease states and the function of organs. ... Human red blood cells Red blood cells are the most common type of blood cell and the vertebrate bodys principal means of delivering oxygen from the lungs or gills to body tissues via the blood. ... A bone marrow biopsy is a medical procedure used as part of a test in the diagnosis of several conditions including leukemia. ...


Pathology

An LCH patient produces an excessive number of histiocytes, which cluster around one or more organs of the body, attacking the very same tissues they were assigned to defend from infection. It is not known what causes this abnormal behavior.


Treatment

Langerhans cell histiocytosis is usually treated much like a cancer using chemotherapy, radiation therapy, and/or surgery. Chemotherapy is by far the most common treatment, with most lesions responding either to chemotherapy or surgery. The most effective drugs used in treatment are Vincristine, Vinblastine, Etoposide and glucocorticoids. Radiation therapy on bone lesions is used in rare cases where these do not respond to chemotherapy or surgery. No effective treatment is known when lesions are found in multiple places and include life-supporting organs such as the liver and bone marrow, although chemotherapy will usually be tried. Cancer is a class of diseases or disorders characterized by uncontrolled division of cells and the ability of these cells to spread, either by direct growth into adjacent tissue through invasion, or by implantation into distant sites by metastasis (where cancer cells are transported through the bloodstream or lymphatic system). ... Chemotherapy is the use of chemical substances to treat disease. ... Clinac 2100 C100 accelerator Radiation therapy (or Radiotherapy) is the medical use of ionizing radiation as part of cancer treatment to control malignant cells (not to be confused with radiology, the use of radiation in medical imaging and diagnosis). ... A cardiothoracic surgeon performs a mitral valve replacement at the Fitzsimons Army Medical Center. ... Vincristine (Oncovin®) is a vinca alkaloid from the Madagascar periwinkle (Catharanthus roseus, formerly Vinca rosea and hence its name). ... Vinblastine is a drug used to treat certain kinds of cancer, including Hodgkins lymphoma, non-small cell lung cancer and breast cancer or testicular cancer. ... Etoposide: chemical structure Etoposide phosphate (Eposin®, Etopophos®, Vepesid®, VP-16®) is an inhibitor of the enzyme topoisomerase II. It is used as a form of chemotherapy for malignancies such as lung cancer, testicular cancer, lymphoma, non-lymphocytic leukemia, and glioblastoma multiforme. ... Glucocorticoids are a class of steroid hormones characterised by an ability to bind with the cortisol receptor and trigger similar effects. ...


Some patients need replacement vasopressin, which is administered by nose drops. Where needed, this treatment is usually required for the remainder of the patient's life. Arginine vasopressin (AVP), also known as argipressin or antidiuretic hormone (ADH), is a human hormone that is mainly released when the body is low on water; it causes the kidneys to conserve water by concentrating the urine and reducing urine volume. ...


Unlike cancer, LCH can spontaneously go into remission. This is quite likely when only a single body part is affected, so in cases where the disease is localised to one body part and the lesions are not endangering the patient (as in the case where they affect a non-weight bearing bone) no treatment may be offered.


The exact cause of this disease is still unknown, nor is there any known cure.


LCH in popular culture

This disease was featured in House episode 3.10, Merry Little Christmas. A house in Pathanapuram, Kerala (India) A house, a structure used for habitation by people, generally has walls and a roof to shelter its enclosed space from precipitation, wind, heat, and cold. ... Merry Little Christmas is the tenth episode of the third season of House, and the fifty-sixth episode overall. ...


See also

Though histiocytosis can refer to any of several specific diseases, the term is generally used to refer to a rare blood disease that is caused by an excess of white blood cells called histiocytes. ... Langerhans cells are immature dendritic cells containing large granules called Birbeck granules. ...

External links

  • The Histiocytosis Association of America
  • Langerhans cell histiocytosis, article by the Sydney Children's Hospital]
  • Dermatlas: Images of Langerhans cell histiocytosis

synd/2224 at Who Named It Who Named It is a Norwegian database of several thousand eponymous medical signs and the doctors associated with their identification. ...


 
 

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