| Histiocytosis Classification and external resources |
|
| ICD-10 | C96.1, D76.0 |
|---|---|
| ICD-9 | 202.3, 277.89 |
| eMedicine | ped/1997 |
| MeSH | D015614 |
In medicine, histiocytosis is an excessive number of histiocytes,[1] 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. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The International Statistical Classification of Diseases and Related Health Problems 10th Revision ( ICD -10) is a coding of diseases and signs symptoms abnormal findings The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. eMedicine is an online clinical medical knowledge base that was founded in 1996 by Scott Plantz and Richard Lavely two medical doctors Medical Subject Headings ( MeSH) is a huge Controlled vocabulary (or metadata system for the purpose of indexing journal articles and books Medicine is the art and science of healing It encompasses a range of Health care practices evolved to maintain and restore Human Health by the A histiocyte is a cell that is part of the human Immune system. Macrophages ( Greek: "big eaters" from makros "large" + phagein "eat" ( Mø) are cells within the tissues that Occasionally and confusingly it is sometimes used to refer to individual diseases.
According to the Histiocytosis Association of America, 1 in 200,000 children in the United States are born with histiocytosis each year. The United States of America —commonly referred to as the [2] HAA also states that most of the people diagnosed with histiocytosis are children under the age of 10. The University of California, San Francisco, states that the disease usually occurs from birth to age 15. [3]
Histiocytosis (and malignant histiocytosis) are both important in veterinary as well as human pathology. Malignant histiocytosis is a hereditary disease found in the Bernese Mountain Dog characterized by histiocytic infiltration of the lungs and Lymph nodes
Contents |
Presentation
Histiocytosis is frequently associated with diabetes insipidus, even after several years of diagnosis and successful therapy. Diabetes insipidus ( DI) is a condition characterized by excretion of large amounts of severely diluted Urine, which cannot be reduced when fluid intake
Classification, and relationships to other conditions
There are competing systems for classifying histiocytoses. According to the 1999 classification proposed by the World Health Organization, they can be divided into three categories. [4][5]. However, the classifications in ICD10 and MeSH are slightly different, as shown below:
| Name | WHO | ICD10 | MeSH |
| Langerhans cell histiocytosis (LCH) | I | D76.0 | Langerhans-cell histiocytosis |
| Juvenile xanthogranuloma (JXG) | II | D76.3 | non-Langerhans-cell histiocytosis |
| Hemophagocytic lymphohistiocytosis (HLH) | II | D76.1 | non-Langerhans-cell histiocytosis |
| Niemann-Pick disease | - | E75.2 | non-Langerhans-cell histiocytosis |
| Sea-blue histiocyte syndrome | - | - | non-Langerhans-cell histiocytosis |
| Acute monocytic leukemia | III | C93.0 | malignant histiocytic disorders |
| Malignant histiocytosis | III | C96.1 | malignant histiocytic disorders |
| Erdheim-Chester disease | - | C96.1 | malignant histiocytic disorders |
Types of LCH have also been known as "Eosinophilic Granuloma", "Hand-Schuller-Christian Disease", "Letterer-Siwe Disease", and "Histiocytosis X". The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases Medical Subject Headings ( MeSH) is a huge Controlled vocabulary (or metadata system for the purpose of indexing journal articles and books Langerhans Cell Histiocytosis (LCH is a rare Disease involving clonal proliferation of Langerhans cells, abnormal cells deriving from Bone marrow Juvenile xanthogranuloma is a form of Histiocytosis, classified as "non-Langerhans cell histiocytosis" or more specifically "type 2" Hemophagocytic lymphohistiocytosis (HLH is an unusual syndrome characterized by Fever, Splenomegaly, Jaundice, and the pathologic finding of Hemophagocytosis Niemann-Pick Disease is one of a group of Lysosome storage diseases that affect Metabolism and that are caused by genetic mutations E00-E35 - Endocrine diseases (E00-E07 Thyroid gland / Thyroid hormone ( Congenital iodine-deficiency syndrome ( Sea-blue histiocyte syndrome is a Sphingolipid disorder (a Sphingolipidosis) Acute monocytic leukemia ( AMoL, or AML-M5) is considered a type of Acute myeloid leukemia. Malignant histiocytosis is a hereditary disease found in the Bernese Mountain Dog characterized by histiocytic infiltration of the lungs and Lymph nodes Erdheim-Chester disease (also known as Erdheim-Chester syndrome or polyostotic sclerosing histiocytosis) is a rare disease characterized by the abnormal multiplication (See LCH history for details). Langerhans Cell Histiocytosis (LCH is a rare Disease involving clonal proliferation of Langerhans cells, abnormal cells deriving from Bone marrow
Common treatments
- Chemotherapy
- Cladribine (also known as 2CDA or Leustatin)
- Etoposide
- Vinblastine (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,[1] or the Histiocytosis Research Trust. Cladribine is a drug commonly used to treat Hairy cell leukemia (leukemic reticuloendotheliosis Etoposide phosphate (brand names Eposin, Etopophos, Vepesid, VP-16) is an inhibitor of the Enzyme Topoisomerase II. Vinblastine is an anti-mitotic drug used to treat certain kinds of Cancer, including Hodgkin's lymphoma, non-small cell Lung cancer
References
- ^ Stedman's Medical Dictionary. URL: http://www.emedicine.com/asp/dictionary.asp?keyword=Histiocytosis. Accessed on: May 24, 2007.
- ^ Disease information at the Histiocytosis Association of America
- ^ Histiocytosis - Signs and Symptoms
- ^ Harris N, Jaffe E, Diebold J, Flandrin G, Muller-Hermelink H, Vardiman J, Lister T, Bloomfield C (1999). "The World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues. Report of the Clinical Advisory Committee meeting, Airlie House, Virginia, November, 1997". Ann Oncol 10 (12): 1419–32. doi:. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document. PMID 10643532.
- ^ eMedicine - Histiocytosis : Article by Cameron K Tebbi
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