WordNet

a blood disease characterized by an abnormal multiplication of macrophages

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2018/01/11 12:35:43」(JST)

wiki en

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 of America, 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

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

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

Organizations

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

^ 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

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

1. ランゲルハンス細胞組織球症の臨床症状、病理学的特徴、および診断 clinical manifestations pathologic features and diagnosis of langerhans cell histiocytosis
2. ランゲルハンス細胞組織球症の治療 treatment of langerhans cell histiocytosis
3. 肺ランゲルハンス細胞組織球症 pulmonary langerhans cell histiocytosis
4. 小児および思春期におけるランゲルハンス細胞組織球症（好酸球性肉芽腫）骨病変 langerhans cell histiocytosis eosinophilic granuloma of bone in children and adolescents
5. 小児における末梢のリンパ節腫脹 peripheral lymphadenopathy in children etiology

English Journal

BRAFV600E-mutation is invariably present and associated to oncogene-induced senescence in Erdheim-Chester disease.

Cangi MG1, Biavasco R2, Cavalli G3, Grassini G1, Dal-Cin E1, Campochiaro C3, Guglielmi B4, Berti A3, Lampasona V5, von Deimling A6, Sabbadini MG3, Ferrarini M7, Doglioni C8, Dagna L3.
Annals of the rheumatic diseases.Ann Rheum Dis.2015 Aug;74(8):1596-602. doi: 10.1136/annrheumdis-2013-204924. Epub 2014 Mar 26.
OBJECTIVES: Erdheim-Chester disease (ECD) is a rare form of histiocytosis characterised by uncontrolled chronic inflammation. The oncogenic BRAF(V600E) mutation has been reported in biopsies in 19 out of 37 patients with ECD from the largest published cohort, but never found in the patients' periphe
PMID 24671772

18F-FDG PET/CT Follow-up of Rosai-Dorfman Disease.

Albano D1, Bosio G, Bertagna F.
Clinical nuclear medicine.Clin Nucl Med.2015 Aug;40(8):e420-2. doi: 10.1097/RLU.0000000000000853.
Rosai-Dorfman disease (RDD) or sinus histiocytosis is a rare histiocytic disorder characterized by massive lymphadenopathy and with extranodal involvement in 25% to 43% of cases. The clinical course of RDD is unpredictable with episodes of exacerbation and remissions that can last many years, and tr
PMID 26053720

Suspected early onset of congenital Langerhans cell histiocytosis involving ectopic cervical thymus and mediastinal thymus, simultaneously.

Kanamitsu K1, Washio K1, Oda M1, Noda T2, Oyama T2, Tanimoto T2, Tanaka T3, Ida K4, Shimada A1.
Pediatric blood & cancer.Pediatr Blood Cancer.2015 Aug;62(8):1491-2. doi: 10.1002/pbc.25496. Epub 2015 Mar 25.
PMID 25810002

Japanese Journal

肉芽腫性肺疾患の画像診断

山根彩,一色彩子,市川太郎,田島廣之
呼吸器内科 = Respiratory medicine 35(2), 167-172, 2019-02
NAID 40021814037

ランゲルハンス細胞組織球症(LCH) (特集肉芽腫形成と呼吸器疾患 : 診断と治療の最前線) -- (原因不明の肉芽腫症)

巽浩一郎,井上義一
呼吸器内科 = Respiratory medicine 35(2), 142-149, 2019-02
NAID 40021814004

Remission of Congenital Multi-system Type Langerhans Cell Histiocytosis with Chemotherapy

Tamefusa Kosuke,Ishida Hisashi,Washio Kana,Ishida Toshiaki,Morita Hirosuke,Shimada Akira
Acta Medica Okayama 73(1), 61-65, 2019-02
… Patients with multi-system (MS)-type langerhans cell histiocytosis (LCH) show poor outcomes, especially congenital MS LCH cases were shown in high mortality rate. …
NAID 120006559388

Related Pictures

Histiocytosis, Langerhans-Cell; Histiocytosis X; Langerhans-Cell Granulomatosis Langerhans cell histiocytosis - Wikipedia Pin Langerhans-cell-histiocytosis on Pinterest Histiocytosis, Sinus; Destombes-Rosai-Dorfman Syndrome; Rosai-Dorfman Disease Histiocytosis - Symptoms, Treatment, Medications

★リンクテーブル★

リンク元	「組織球症」「組織球増殖症」「ヒスチオサイトーシス」
拡張検索	「familial hemophagocytic lymphohistiocytosis」「histiocytosis X」「Langerhans cell histiocytosis」「Langerhans-cell histiocytosis」

「組織球症」

Histiocytosis
Classification and external resources
Specialty	hematology
ICD-10	C96.1, D76.0
ICD-9-CM	202.3, 277.89
MedlinePlus	000068
eMedicine	ped/1997
MeSH	D015614
	[edit on Wikidata]

　　[★]

英: histiocytosis
同: マクロファージ増殖症 macrophage hyperplasia
関: 組織球、組織球増殖症、ヒスチオサイトーシス

「組織球増殖症」

　　[★]

英: histiocytosis
関: 組織球症、ヒスチオサイトーシス

「ヒスチオサイトーシス」

　　[★]

英: histiocytosis
関: 組織球増殖症、組織球症

「familial hemophagocytic lymphohistiocytosis」

　　[★] 家族性血球貪食性リンパ組織球症 FHL FHLH

「histiocytosis X」

　　[★] ランゲルハンス細胞組織球症、組織球症X

「Langerhans cell histiocytosis」

　　[★] ランゲルハンス細胞組織球症 LCH

「Langerhans-cell histiocytosis」