Histiocytosis

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Histiocytosis

In medicine

Medicine

Medicine is the art and science of healing. It encompasses a range of health care practices evolved to maintain and restore health by the prevention and treatment of illness....
, histiocytosis is an excessive number of histiocyte

Histiocyte

A histiocyte is a Cell that is part of the mononuclear phagocytic system . The mononuclear phagocytic system is part of the organism's immune system....
s, that is, an excessive number of tissue macrophages, and is typically used to refer to a group of rare diseases which share this as a characteristic. Occasionally and confusingly, it is sometimes used to refer to individual diseases.

The histiocytes may attack skin, bone, muscles, and other important organs, including the liver, lung, spleen, and hematopoietic system.

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Encyclopedia

In medicine

Medicine

Histiocyte

Cancer

Cancer is a class of diseases in which a group of cell display uncontrolled growth , invasion , and sometimes metastasis . These three malignant properties of cancers differentiate them from benign tumors, which are self-limited, do not invade or metastasize....
, and treatment often involves radiation and chemotherapy

Chemotherapy

Chemotherapy, in its most general sense, refers to treatment of disease by chemicals that kill cells, specifically those of micro-organisms or cancer....
.

According to the Histiocytosis Association of America, 1 in 200,000 children in the United States

United States

The United States of America is a Federal government constitutional republic comprising U.S. state and a federal district. The country is situated mostly in central North America, where its Contiguous United States and Washington, D.C., the Capital districts and territories, lie between the Pacific Ocean and Atlantic Oceans, Borders of the U...
are born with histiocytosis each year. HAA also states that most of the people diagnosed with histiocytosis are children under the age of 10. The University of California, San Francisco

University of California, San Francisco

The University of California, San Francisco is one of the world's leading centers of health sciences research, patient care, and education. UCSF's medical, pharmacy, dentistry, nursing, and graduate schools are among the top health science professional schools in the world....
, states that the disease usually occurs from birth to age 15.

Histiocytosis (and malignant histiocytosis

Malignant histiocytosis

Malignant histiocytosis is a rare hereditary disease found in the Bernese Mountain Dog characterized by histiocyte infiltration of the lungs and lymph nodes....
) are both important in veterinary as well as human pathology.

Presentation

Histiocytosis is frequently associated with diabetes insipidus

Diabetes insipidus

Diabetes insipidus is a disease characterized by polydipsia and excretion of large amounts of severely diluted polyuria, with reduction of fluid intake having no effect on the latter....
, even after several years of diagnosis and successful therapy.

Classification, and relationships to other conditions

There are competing systems for classifying histiocytoses. According to the 1999 classification proposed by the World Health Organization

World Health Organization

The World Health Organization is a specialized agency of the United Nations that acts as a coordinating authority on international public health....
, they can be divided into three categories.. However, the classifications in ICD10 and MeSH are slightly different, as shown below:

Name	WHO	ICD10	MeSH Mesh Mesh consists of semi-permeable barrier made of connected strands of metal, fiber, or other flexible/ductile material. Mesh is similar to spider web or Net in that it has many attached or woven strands.... \|- \| Langerhans cell histiocytosis Langerhans cell histiocytosis Langerhans cell histiocytosis is a rare disease involving clonal proliferation of Langerhans cells, abnormal cell s deriving from bone marrow and capable of migrating from skin to lymph nodes.... (LCH)	I		- \| Juvenile xanthogranuloma Juvenile xanthogranuloma Juvenile xanthogranuloma is a form of histiocytosis, classified as "non-Langerhans cell histiocytosis", or more specifically, "type 2".... (JXG)	II		- \| Hemophagocytic lymphohistiocytosis Hemophagocytic lymphohistiocytosis Hemophagocytic lymphohistiocytosis, abbreviated HLH and also known as hemophagocytic syndrome, is an uncommon haematologic disorder that, typically, clinically manifests as fever, splenomegaly and jaundice, with medical laboratory findings of lymphocytosis and histiocytosis, and has the pathologic finding of hemophagocytosis.... (HLH)	II		- \| Niemann-Pick disease Niemann-Pick disease Niemann-Pick disease refers to a group of fatal inherited metabolic disorders that are included in the larger family of lysosomal storage diseases ....	-		- \| Sea-blue histiocyte syndrome Sea-blue histiocyte syndrome Sea-blue histiocyte syndrome is a sphingolipid disorder .It can be associated with the gene Apolipoprotein ESea-blue histiocyte syndrome is seen in patients receiving fat emulsion as a part of long-term parental nutrition for intestinal failure....	-	-	- \| Acute monocytic leukemia Acute monocytic leukemia Acute monocytic leukemia is considered a type of acute myeloid leukemia....	III		- \| Malignant histiocytosis Malignant histiocytosis Malignant histiocytosis is a rare hereditary disease found in the Bernese Mountain Dog characterized by histiocyte infiltration of the lungs and lymph nodes....	III		- \| Erdheim-Chester disease Erdheim-Chester disease Erdheim-Chester disease is a rare disease characterized by the abnormal multiplication of a specific type of white blood cells called histiocytes, or tissue macrophages ....	-		malignant histiocytic disorders

Types of LCH have also been known as "Eosinophilic Granuloma", "Hand-Schuller-Christian Disease", "Letterer-Siwe Disease", and "Histiocytosis X". (See LCH history for details).

Common treatments

Chemotherapy

Cladribine
Cladribine

Cladribine is a medication used to treat hairy cell leukemia and multiple sclerosis. Its chemical name is 2-chlorodeoxyadenosine .As a purine analog, it is a synthetic antineoplastic agent that also suppresses the immune system....
(also known as 2CDA or Leustatin)
Etoposide
Etoposide

Etoposide phosphate is an inhibitor of the enzyme topoisomerase II. It is used as a form of chemotherapy for malignancies such as Ewing's sarcoma, lung cancer, testicular cancer, lymphoma, non-lymphocytic leukemia, and glioblastoma multiforme....
Vinblastine
Vinblastine

Vinblastine is an mitotic inhibitor medication used to treat certain kinds of cancer, including Hodgkin's lymphoma, non-small cell lung cancer, breast cancer, head and neck cancer, and testicular cancer....
(Velban)

Organizations

Patients and families can gain some support by contacting patient organisations such as the Histiocytosis Association of America, which has several stable and proven treatment protocols available only for physicians, or the Histiocytosis Research Trust.