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In medicine, histiocytosis refers to an excessive number of histiocytes,^[1] (tissue macrophages), and is typically used to refer to a group of rare diseases which share this as a characteristic. Occasionally and confusingly, the term "histiocytosis" is sometimes used to refer to individual diseases.

According to the Histiocytosis Association, 1 in 200,000 children in the United States are born with histiocytosis each year.^[2] HAA also states that most of the people diagnosed with histiocytosis are children under the age of 10, although the disease can afflict adults. 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.

Classification, and relationships to other conditions[edit]

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	II	E75.2	non-Langerhans-cell histiocytosis
Sea-blue histiocytosis	II	-	non-Langerhans-cell histiocytosis
Acute monocytic leukemia	III	C93.0	malignant histiocytic disorders
Malignant histiocytosis	III	C96.1	malignant histiocytic disorders
Erdheim–Chester disease	II	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". (See Langerhans cell histiocytosis for details).

Alternatively, histiocytoses may be divided into the following groups:^[6]^:714-724

X-type histiocytoses
Non-X histiocytoses

Common treatments[edit]

Chemotherapy
- Cladribine (also known as 2CDA or Leustatin)
- Etoposide
- Vinblastine (Velban)

Organizations[edit]

Patients and families can gain support and educational materials from the Histiocytosis Association, or the Histiocytosis Research Trust. Information concerning histiocytosis and clinicians located in European countries may be found in many languages at the web portal of Euro Histio Net (EHN). This is a project funded by the European Union, co-ordinated by Dr. Jean Donadieu, APHP, Paris, FRANCE. Additional information about Erdheim-Chester Disease can be obtained from the ECD Global Alliance.

The Histiocyte Society, a nonprofit organization, is a group of more than 200 physicians and scientists from around the world committed to improving the lives of patients with histiocytic disorders by conducting clinical and laboratory research into the causes and treatment of this disease. The Society has instituted several clinical trials and treatment plans.

References[edit]

^ Histiocytosis at eMedicine Dictionary
^ 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:10.1023/A:1008375931236. PMID 10643532.
^ Histiocytosis at eMedicine
^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.

UpToDate Contents

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1. 血球貪食リンパ組織球の臨床的特徴および診断 clinical features and diagnosis of hemophagocytic lymphohistiocytosis
2. 血球貪食性リンパ組織球症（HLH）の治療および予後 treatment and prognosis of hemophagocytic lymphohistiocytosis
3. エプスタインバーウイルス（EBウイルス）感染の臨床的特徴および治療 clinical manifestations and treatment of epstein barr virus infection
4. 小児における末梢のリンパ節腫脹 peripheral lymphadenopathy in children etiology
5. 小児における急性肝不全：病因および評価 acute liver failure in children etiology and evaluation

English Journal

Secondary Hemophagocytosis in 3 Patients With Organic Acidemia Involving Propionate Metabolism.

Gokce M, Unal O, Hismi B, Gumruk F, Coskun T, Balta G, Unal S, Cetin M, Kalkanoglu-Sivri HS, Dursun A, Tokatl? A.Source1Department of Pediatric Hematology, Hacettepe Medical Faculty, Ankara, Turkey.
Pediatric hematology and oncology.Pediatr Hematol Oncol.2011 Oct 4. [Epub ahead of print]
Hemophagocytic lymphohistiocytosis (HLH) may develop secondary to infections, malignancies, immune deficiency syndromes, and rheumatologic and metabolic disorders. Associations between HLH and inborn errors of metabolism, including lysinuric protein intolerance, multiple sulfatase deficiency, galact
PMID 21970506

The expanding spectrum of hemophagocytic lymphohistiocytosis.

Filipovich AH.SourceCincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio, USA.
Current opinion in allergy and clinical immunology.Curr Opin Allergy Clin Immunol.2011 Oct 3. [Epub ahead of print]
PURPOSE OF REVIEW: Hemophagocytic lymphohistiocytosis (HLH) is more widely recognized by clinicians. No longer viewed as a disorder of young children, adult patients are now being identified and treated. In this review, I summarize clinical features of patients with recently identified genetic cause
PMID 21971331

Japanese Journal

EBウイルス関連血球貪食症候群--感染標的細胞と治療選択 (AYUMI 血球貪食症候群の発症機構に迫る)

土居岳彦,大賀正一
医学のあゆみ 238(11), 1053-1057, 2011-09-10
NAID 40018973513

家族性血球貪食症候群の病態と診断--細胞障害活性による免疫応答の調節 (AYUMI 血球貪食症候群の発症機構に迫る)

八角高裕
医学のあゆみ 238(11), 1048-1052, 2011-09-10
NAID 40018973512

Related Pictures

★リンクテーブル★

リンク元	「リンパ組織球症」「リンパ組織球増多症」「リンパ組織球増殖症」
拡張検索	「familial hemophagocytic lymphohistiocytosis」「hemophagocytic lymphohistiocytosis」