Langerhans-cell Histiocytosis malady

NIH Rare Diseases: Langerhans cell histiocytosis (LCH) is a disorder that primarily affects children, but is also found in adults of all ages. People with LCH produce too many Langerhans cells or histiocytes, a form of white blood cell found in healthy people that is supposed to protect the body from infection. In people with LCH, these cells multiply excessively and build up in certain areas of the body, causing tumors called granulomas to form. The symptoms vary among affected individuals, and the cause of LCH is unknown. In most cases, this condition is not life-threatening. Some people do experience life-long problems associated with LCH.³⁰

MalaCards: Langerhans-cell Histiocytosis, also known as histiocytosis x, is related to non-langerhans-cell histiocytosis and rosai-dorfman disease. An important gene associated with Langerhans-cell Histiocytosis is F13A1 (coagulation factor XIII, A1 polypeptide), and among its related pathways are all-trans-Retinoic Acid Mediated Apoptosis and Immune response_CD16 signaling in NK cells. The drug thalidomide and the compounds cyclophosphamide and ivig have been mentioned in the context of this disorder. Affiliated tissues include bone marrow, lymph node and spleen, and related mouse phenotypes are respiratory system and endocrine/exocrine gland.

Disease Ontology: A disease of metabolism and histiocytic and dendritic cell cancer and histiocytosis and interstitial lung disease that results in the proliferation of langerhans cells.⁶

Genetics Home Reference: Langerhans cell histiocytosis is a disorder in which excess immune system cells called Langerhans cells build up in the body. Langerhans cells, which help regulate the immune system, are normally found throughout the body, especially in the skin, lymph nodes, spleen, lungs, liver, and bone marrow. In Langerhans cell histiocytosis, excess immature Langerhans cells usually form tumors called granulomas. However, Langerhans cell histiocytosis is not generally considered to be a form of cancer.¹⁷

Wikipedia: Langerhans cell Histiocytosis (LCH) is a rare disease involving clonal proliferation of Langerhans...⁴⁴ more...

Aliases & Descriptions:

langerhans-cell histiocytosis 6 8 histiocytosis x 6 30 17 32 langerhans cell histiocytosis 30 17 32 eosinophilic granuloma 30 32 lch 30 17 langerhan's cell histiocytosis (disorder) 6

hand-schüller-christian syndrome 30 langerhans cell granulomatosis 6 histiocytosis, langerhans-cell 43 hashimoto-pritzger disease 17 letterer-siwe disease 30 hashimoto disease 43

Diseases related to langerhans-cell histiocytosis by text searches and GeneDecks gene sharing:

(show top 50)    (show all 904)

id	Related Disease	Score	Top Affiliating Genes
1	non-langerhans-cell histiocytosis	35.2	CD163, F13A1, ACTC1
2	rosai-dorfman disease	32.8	S100A1, S100B, FSCN1, CD163, CTSD
3	juvenile xanthogranuloma	32.7	KIT, S100B, FSCN1, F13A1
4	langerhans cell sarcoma	31.7	S100B, CD1A, CD207
5	letterer-siwe disease	30.9	RARA, LIF, S100B, FSCN1, CD1A, CD207
6	erdheim-chester disease	30.4	S100B, IL1B, CCR6, CCR7, SST
7	fibromatosis	30.2	KIT, S100A1, CD163, MKI67, IL1B, F13A1
8	diabetes insipidus	29.8	S100B, AVP, CD1A, INS, TRH, CRH
9	idiopathic pulmonary fibrosis	29.6	S100A1, IL1B, IL1R1, IL1RN, CCL22, CCR7
10	kimura disease	29.3	KIT, IL4, IFNA1
11	protein-losing enteropathy	28.5	INS, TP53, TNF, SST
12	periodontitis	27.1	IL10, IL17A, IL1B, IL1R1, IL1RN, TNF
13	eosinophilic pneumonia	26.5	IL10, IL1B, IL1RN, IL4, CCL22, IFNG
14	aggressive periodontitis	26.3	CD1A, IL10, IL1B, IL1RN, IL4, IFNG
15	sinusitis	25.7	S100A1, AVP, CD40, MKI67, INS, IL17A
16	sarcoidosis	24.7	CLEC12A, CD207, CD68, IL10, IL1B, IL1R1
17	histiocytosis	23.9	NCAM1, KIT, KITLG, RARA, LIF, CLEC12A
18	pulmonary fibrosis	23.8	CLEC12A, S100A1, CFLAR, CD207, IL10, IL11
19	hypothyroidism	22.6	RARA, LIF, AVP, INS, IL10, IL2
20	autoimmune thyroiditis	22.2	CD40, FLT3LG, INS, IL10, IL1B, IL1RN
21	lichen planus	21.9	CD1A, CD207, CD68, MKI67, INS, IL10
22	pneumonia	20.4	RARA, LIF, S100A1, AVP, CD40, ITPA
23	graves' disease	20.3	KITLG, CD1A, CD1C, CD40, MKI67, INS
24	fibrosis	19.6	KIT, KITLG, RARA, LIF, CLEC12A, S100A1
25	thyroiditis	18.9	NCAM1, KIT, KITLG, RARA, LIF, S100A1
26	leukemia	16.8	NCAM1, KIT, KITLG, RARA, LIF, CLEC12A
27	follicular dendritic cell tumor	13.8	S100B, FSCN1, CD68, ACTC1
28	fibrous histiocytoma	13.8	S100B, FSCN1, CD163, F13A1, ACTC1
29	ossifying fibromyxoid tumor	13.8	S100A1, S100B, ACTC1
30	inflammatory myofibroblastic tumor	13.7	S100A1, MKI67, F13A1, ACTC1
31	ossifying fibroma	13.7	S100A1, F13A1, ACTC1
32	cutaneous fibrous histiocytoma	13.7	KITLG, S100A1, S100B, F13A1, ACTC1
33	giant cell tumor	13.7	S100B, F13A1, TNFRSF11B, ACTC1, CSF1
34	cutaneous leiomyosarcoma	13.7	KIT, S100B, ACTC1
35	pulmonary vein stenosis	13.7	MKI67, ACTC1, PECAM1
36	granular cell tumor	13.6	S100A1, S100B, CD68, MKI67, F13A1
37	rectum sarcomatoid carcinoma	13.6	S100A1, S100B, MKI67, ACTC1
38	cystic fibrosis	13.6	RARA, CTSD, PRL
39	polymorphous low-grade adenocarcinoma	13.6	KIT, S100A1, S100B, ACTC1
40	malignant mixed mullerian tumor	13.6	KIT, S100A1, S100B, ACTC1
41	endometrial stromal sarcoma	13.6	KIT, ACTC1, CSF1
42	malignant peripheral nerve sheath tumor	13.6	KIT, S100A1, S100B, ACTC1
43	neurilemmoma	13.5	KIT, S100A1, S100B, F13A1, ACTC1
44	cutaneous mastocytosis	13.5	KIT, KITLG, RARA
45	angiofibroma	13.5	KIT, S100B, F13A1, ACTC1
46	sarcomatoid renal cell carcinoma	13.5	KIT, S100A1, ACTC1
47	adenosarcoma	13.5	KIT, MKI67, ACTC1
48	petrositis	13.5	AVP, CRH, PRL
49	neurogenic diabetes insipidus	13.5	AVP, CRH, PRL
50	isolated acth deficiency	13.5	TRH, CRH, PRL

Approved drugs:

Drug clinical trials:

Inferred drug relations via UMLS/NDF-RT:

^43 28 thalidomide

MalaCards organs/tissues related to langerhans-cell histiocytosis:

²²

MGI Mouse Phenotypes related to langerhans-cell histiocytosis:

²⁵ (show all 24)

id	Description	MGI Source Accession	Score	Top Affiliating Genes
1	respiratory system phenotype	MP:0005388	10.1	ADA, CRH, PTPN6, F13A1, ITPA, LIF
2	endocrine/exocrine gland phenotype	MP:0005379	9.6	PTPN6, TRH, CASP8, IFNG, IL2
3	craniofacial phenotype	MP:0005382	9.4	CSF2, CSF1, NOTCH1, IL17A, KITLG, KIT
4	embryogenesis phenotype	MP:0005380	9.2	KIT, CFLAR, FADD, NOTCH1, PRL, CSF2
5	renal/urinary system phenotype	MP:0005367	9.0	NOTCH1, OAT, INS, AVP, RARA, KIT
6	hematopoietic system phenotype	MP:0005397	8.8	KIT, CSF2, PML, ADA, NOTCH1, PTPN6
7	no phenotypic analysis	MP:0003012	8.3	PML, SST, TP53, FADD, IL17A, INS
8	reproductive system phenotype	MP:0005389	7.9	CSF2, CSF1, PRL, PTPN6, TNF, IFNG
9	digestive/alimentary phenotype	MP:0005381	7.9	PTPN6, SST, CTSD, NOTCH1, SLC18A2, PECAM1
10	liver/biliary system phenotype	MP:0005370	7.8	ADA, PRL, TP53, IFNG, IL2, IL1R1
11	nervous system phenotype	MP:0003631	7.5	TRH, PTPN6, OAT, CTSD, CRH, PRL
12	skeleton phenotype	MP:0005390	7.4	TNFRSF11B, PTPN6, NOTCH1, CSF1, CSF2, PECAM1
13	muscle phenotype	MP:0005369	7.1	CASP8, TP53, TNF, NOTCH1, ACTC1, CSF1
14	mortality/aging	MP:0010768	6.9	TNFRSF11B, PTPN6, OAT, CTSD, CSF1, CSF2
15	immune system phenotype	MP:0005387	6.9	KIT, TNF, PTPN6, CTSD, CRH, PRL
16	normal phenotype	MP:0002873	6.8	SST, CRH, NOTCH1, PML, SLC18A1, ADA
17	tumorigenesis	MP:0002006	6.5	PML, CSF2, PRL, NOTCH1, TNF, TP53
18	growth/size phenotype	MP:0005378	6.3	TNFRSF11B, PTPN6, SST, CRH, NOTCH1, ACTC1
19	behavior/neurological phenotype	MP:0005386	6.2	IL2, IFNG, SST, OAT, CTSD, PRL
20	vision/eye phenotype	MP:0005391	6.2	IFNG, TP53, TNF, OAT, CTSD, NOTCH1
21	integument phenotype	MP:0010771	5.5	CASP8, TP53, TNF, PTPN6, CRH, NOTCH1
22	homeostasis/metabolism phenotype	MP:0005376	5.1	KIT, KITLG, CFLAR, AVP, CD40, CTSD
23	cardiovascular system phenotype	MP:0005385	4.6	IFNG, F13A1, CASP8, TP53, TNF, TNFRSF11B
24	cellular phenotype	MP:0005384	4.3	NCAM1, KIT, KITLG, RARA, LIF, CFLAR

Articles related to langerhans-cell histiocytosis:

(show top 50)    (show all 60)

id	Title	Authors	Year	Affiliating Genes
1	Tyrosine phosphatase SHP-1 is expressed higher in mul tisystem than in single-system Langerhans cell histiocytosis by immunohistochem istry. (21604205)	Murakami I.... Yoshino T.	2011	PTPN6
2	A role for ITPA variants in the clinical course of pu lmonary Langerhans' cell histiocytosis? (20930204)	Bakker J.A.... Drent M.	2010	ITPA
3	Importance of multi-lineage hematologic involvement a nd hypoalbuminemia at diagnosis in patients with 'risk-organ' multi-s ystem Langerhans cell histiocytosis. (20418784)	Braier J.L.... Scopinaro M.	2010	ALB
4	Fibroblastic rheumatism: fibromatosis rather than non -Langerhans cell histiocytosis. (19615014)	Kluger N.... Fraitag S.	2010	F13A1, ACTC1, CD163
5	Interleukin-17A is not expressed by CD207(+) cells in Langerhans cell histiocytosis lesions. (19424201)	Allen C.E.... McClain K.L.	2009	IL17A
6	Histiocyte-rich pleomorphic mastocytoma: an uncommon variant mimicking juvenile xanthogranuloma and Langerhans cell histiocytosis. (19602070)	Tran D.T.... Argenyi Z.B.	2009	KIT
7	Solitary Langerhans cell histiocytosis orbital lesion : case report and review of the literature. (18936863)	Ulivieri S.... Filosomi G.	2008	CD1A
8	Aggressive Langerhans cell histiocytosis following T-ALL: clonally related neoplasms with persistent expression of constitutively active NOTCH1. (17874453)	Rodig S.J.... Aster J.C.	2008	NOTCH1
9	Immunohistochemical expression of Langerin in Langerhans cell histiocytosis and non-Langerhans cell histiocytic disorders. (18277880)	Lau S.K.... Weiss L.M.	2008	CD1A, CD207
10	Clinical and immunohistochemical evaluation of the vulvar Langerhans cell histiocytosis. (19076626)	Bongiorno M.R.... AricA^ M.	2008	CD207
11	IL-1 beta and PGE2 levels are increased in the saliva of children with Langerhans cell histiocytosis. (18647218)	Preliasco V.F.... Sterin-Borda L.	2008	IL1B
12	Immunohistochemical analysis of langerin in langerhans cell histiocytosis and pulmonary inflammatory and infectious diseases. (17527085)	Sholl L.M.... Padera R.F.	2007	CD1A, CD207
13	Expansion of regulatory T cells in patients with Langerhans cell histiocytosis. (17696642)	Senechal B.... Geissmann F.	2007	IL10
14	Elevated serum levels of the decoy receptor osteoprotegerin in children with langerhans cell histiocytosis. (16439593)	Rosso D.A.... Fadeel B.	2006	TNFRSF11B
15	Clinical spectrum of cutaneous Langerhans' cell histiocytosis mimicking various diseases. (16585988)	Querings K.... Balda B.R.	2006	CD207
16	High serum values of soluble CD154, IL-2 receptor, RANKL and osteoprotegerin in Langerhans cell histiocytosis. (16358318)	Ishii R.... Imashuku S.	2006	TNFRSF11B
17	Specific polymorphisms of cytokine genes are associated with different risks to develop single-system or multi-system childhood Langerhans cell histiocytosis. (16487180)	De Filippi P.... Arico M.	2006	IL4, IFNG
18	Expression of CD56 antigen in Langerhans cell histiocytosis associated with T-lymphoblastic lymphoma in a same lymph node. (16228226)	Ko Y.H.... Kim Y.	2006	NCAM1
19	Solitary cutaneous dendritic cell tumor in a child: role of dendritic cell markers for the diagnosis of skin Langerhans cell histiocytosis. (16243135)	Weiss T.... Weiss J.M.	2005	CD1A, CD207
20	Immunohistochemical detection of the apoptosis-related proteins FADD, FLICE, and FLIP in Langerhans cell histiocytosis. (15956881)	Bank M.I.... Petersen B.L.	2005	CASP8, FADD, CFLAR
21	A case of pulmonary langerhans cell histiocytosis presenting disappearance of coalescing air wall cysts after smoking cessation (16050471)	Okubo F.... Kumamoto T.	2005	CD68
22	DOR-1, A novel CD10+ stromal cell line derived from progressive Langerhans cell histiocytosis of bone. (15390308)	Gogusev J.... Jaubert F.	2005	CD1A, CD207
23	Increased blood myeloid dendritic cells and dendritic cell-poietins in Langerhans cell histiocytosis. (15728521)	Rolland A.... Palucka A.K.	2005	KITLG, FLT3LG, CSF1
24	The vesicular monoamine transporter 2 (VMAT2) is expressed by normal and tumor cutaneous mast cells and Langerhans cells of the skin but is absent from Langerhans cell histiocytosis. (15150286)	Anlauf M.... Weihe E.	2004	SLC18A2, SLC18A1
25	Diagnostic relevance of Langerin detection in cells from bronchoalveolar lavage of patients with pulmonary Langerhans cell histiocytosis, sarcoidosis and idiopathic pulmonary fibrosis. (14722767)	Smetana K.... Gabius H.J.	2004	CD207, CLEC12A
26	Tumor necrosis factor, interleukin 11, and leukemia inhibitory factor produced by Langerhans cells in Langerhans cell histiocytosis. (15543003)	Andersson By U.... Henter J.I.	2004	TNF, LIF, IL11
27	Sinus histiocytosis with massive lymphadenopathy and Langerhans cell histiocytosis express the cellular adhesion molecule CD31. (12653580)	Slone S.P.... Buchino J.J.	2003	PECAM1
28	Serum levels of interleukin-1 receptor antagonist and tumor necrosis factor-alpha are elevated in children with Langerhans cell histiocytosis. (12794527)	Rosso D.A.... Braier J.L.	2003	TNF, IL1R1, IL1RN
29	Coincident expression of the chemokine receptors CCR6 and CCR7 by pathologic Langerhans cells in Langerhans cell histiocytosis. (12642342)	Fleming M.D.... Rollins B.J.	2003	CCR6, CCR7
30	Langerhans cell histiocytosis: an evaluation of histopathological parameters, demonstration of proliferation by Ki-67 and mitotic bodies. (12716386)	Bank M.I.... Petersen B.L.	2003	MKI67
31	Oral Langerhans cell histiocytosis: antigen-Ki-67 as a indicator for local tumor behaviour. (12820324)	Bartnick A.... Schmelzle R.	2003	MKI67
32	Langerhans cell histiocytosis immunohistochemical expression of fascin, a dendritic cell marker. (12219775)	Pinkus G.S.... Pinkus J.L.	2002	FSCN1
33	p53 expression in biopsies from children with Langerhans cell histiocytosis. (12468914)	Bank M.I.... Petersen B.L.	2002	TP53
34	Langerhans cell histiocytosis of an adult with tumors in liver and spleen. (11379359)	Yagita K.... Tsuchihashi Y.	2001	CD1A
35	Etoposide as the basic and interferon-alpha as the ma intenance therapy for Langerhans cell histiocytosis: a RTC. (11400655)	CuliA8 S.... Primorac D.	2001	IFNA1
36	Pulmonary Langerhans cell Histiocytosis. (11587096)	Harari S.... Comel A.	2001	CD1C
37	Abundant expression of CD40 and CD40-ligand (CD154) in paediatric Langerhans cell histiocytosis lesions. (11044648)	Egeler R.M.... Claassen E.	2000	CD40
38	CD101 expression by Langerhans cell histiocytosis cells. (10692025)	Bouloc A.... Bagot M.	2000	CD101
39	Angiotensin-converting enzyme, transforming growth factor beta(1), and interleukin 11 in the osteolytic lesions of Langerhans cell histiocytosis. (10975922)	Brown R.E.	2000	IL11
40	Langerhans cell histiocytosis of the thyroid gland. A case report. (11015983)	el-Halabi D.A.... Dey P.	2000	S100A1
41	Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy. (10464385)	Santos-Machado T.M.... Odone-Filho V.	1999	ALB
42	Evidence that Langerhans cells in adult pulmonary Langerhans cell histiocytosis are mature dendritic cells: importance of the cytokine microenvironment. (10477625)	Tazi A.... Soler P.	1999	TNF, IL10
43	Dynamic endocrine testing and magnetic resonance imaging in the long-term follow-up of childhood langerhans cell histiocytosis. (9745408)	Maghnie M.... AricA^ M.	1998	PRL, CRH, TRH
44	Monokine expression in Langerhans' cell histiocytosis and sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) (8691347)	Foss H.D.... Stein H.	1996	S100A1
45	Successful treatment of two children with Langerhans' cell histiocytosis with 2'-deoxycoformycin. (8846128)	McCowage G.B.... Kurtzberg J.	1996	ADA
46	Functional defect in cells involved in Langerhans cell histiocytosis. (8534124)	Yu R.C.... Chu A.C.	1995	IL1B
47	Cathepsin D and E co-expression in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and Langerhans' cell histiocytosis: further evidences of a phenotypic overlap between these histiocytic disorders. (8055153)	Paulli M.... Bonetti F.	1994	CTSD
48	Anterior pituitary function and computed tomography/magnetic resonance imaging in patients with Langerhans cell histiocytosis and diabetes insipidus. (8412998)	Broadbent V.... Kendall B.	1993	INS
49	Immunohistochemical detection of granulocyte/macrophage colony-stimulating factor in Langerhans' cell histiocytosis. (7507881)	Emile J.F.... Brousse N.	1993	CSF1
50	A case of hypersensitivity syndrome resembling Langerhans cell histiocytosis during phenobarbital prophylaxis for convulsion. (1291759)	Nagata T.... Mino M.	1992	GPT

Genes related to langerhans-cell histiocytosis according to GeneCards:

(show top 50)    (show all 59)

id	Symbol	Description	Score	GeneCards Section Context
1	F13A1	coagulation factor XIII, A1 polypeptide	11.1	Publications
2	CD207	CD207 molecule, langerin	11.0	Publications
3	CD1A	CD1a molecule	11.0	Publications
4	CD68	CD68 molecule	11.0	Publications
5	CD163	CD163 molecule	11.0	Publications
6	FSCN1	fascin homolog 1, actin-bundling protein (Strongylocentrotus purpuratus)	11.0	Publications
7	ACTC1	actin, alpha, cardiac muscle 1	11.0	Publications
8	CSF1	colony stimulating factor 1 (macrophage)	11.0	Publications
9	S100B	S100 calcium binding protein B	11.0	Publications
10	LIF	leukemia inhibitory factor	11.0	Publications
11	CTSD	cathepsin D	11.0	Publications
12	PML	promyelocytic leukemia	11.0
13	RARA	retinoic acid receptor, alpha	11.0
14	CD40	CD40 molecule, TNF receptor superfamily member 5	11.0	Publications
15	CD1C	CD1c molecule	11.0	Publications
16	CD101	CD101 molecule	11.0	Expressions, Publications
17	CCL22	chemokine (C-C motif) ligand 22	11.0	Expressions
18	FLT3LG	fms-related tyrosine kinase 3 ligand	11.0	Publications
19	S100A1	S100 calcium binding protein A1	11.0	Publications
20	SLC18A1	solute carrier family 18 (vesicular monoamine), member 1	11.0	Publications
21	ITPA	inosine triphosphatase (nucleoside triphosphate pyrophosphatase)	11.0	Publications
22	TRH	thyrotropin-releasing hormone	11.0	Publications
23	IL11	interleukin 11	11.0	Publications
24	SLC18A2	solute carrier family 18 (vesicular monoamine), member 2	11.0	Publications
25	IL17A	interleukin 17A	11.0	Publications
26	OAT	ornithine aminotransferase	11.0
27	FADD	Fas (TNFRSF6)-associated via death domain	11.0	Publications
28	CCR7	chemokine (C-C motif) receptor 7	11.0	Publications
29	CLEC12A	C-type lectin domain family 12, member A	11.0	Publications
30	CCR6	chemokine (C-C motif) receptor 6	11.0	Publications
31	AVP	arginine vasopressin	11.0
32	CFLAR	CASP8 and FADD-like apoptosis regulator	11.0	Publications
33	PTPN6	protein tyrosine phosphatase, non-receptor type 6	11.0	Publications
34	PECAM1	platelet/endothelial cell adhesion molecule 1	11.0	Publications
35	NOTCH1	notch 1	11.0	Publications
36	KITLG	KIT ligand	11.0	Publications
37	ADA	adenosine deaminase	11.0	Publications
38	CRH	corticotropin releasing hormone	11.0	Publications
39	MKI67	antigen identified by monoclonal antibody Ki-67	11.0	Publications
40	TNFRSF11B	tumor necrosis factor receptor superfamily, member 11b	11.0	Publications
41	IL1R1	interleukin 1 receptor, type I	11.0	Publications
42	CASP8	caspase 8, apoptosis-related cysteine peptidase	11.0	Publications
43	IL4	interleukin 4	11.0	Publications
44	SST	somatostatin	11.0	Publications
45	PRL	prolactin	11.0	Publications
46	NCAM1	neural cell adhesion molecule 1	11.0	Publications
47	IFNA1	interferon, alpha 1	11.0	Publications
48	CSF2	colony stimulating factor 2 (granulocyte-macrophage)	11.0	Publications
49	GPT	glutamic-pyruvate transaminase (alanine aminotransferase)	11.0	Publications
50	IL1RN	interleukin 1 receptor antagonist	11.0	Publications

Pathways related to langerhans-cell histiocytosis according to GeneDecks:

(show top 50)    (show all 83)

id	Pathway	Score	Top Affiliating Genes
1	all-trans-Retinoic Acid Mediated Apoptosis36	10.2	CASP8, FADD, CFLAR, RARA
2	Immune response_CD16 signaling in NK cells41	10.1	PTPN6, CSF2
3	IL-15 Signaling and its Primary Biological Effects in Different Immune Cell Types37	10.0	CSF2, IL2
4	RAR-Gamma-RXR-Alpha Degradation36	9.9	CSF2, CSF1, CCL22, IL2, IL11, LIF
5	LDL Oxidation in Atherogenesis36	9.7	CSF1, TNF, IL1B, CD68
6	IL-6 Pathway36	9.7	CSF2, CSF1, IL4, IL11, IL10
7	Amoebiasis20	9.6	IL1B, IL1R1, CSF2
8	Regulation of eIF4 and p70S6K36	9.4	CCL22, IL4, IL2, IL11
9	IL-21 Signaling and its Primary Biological Effects in Different Immune Cell Types37	9.4	IFNG, CCR7, IL4, IL1B, IL17A
10	PGC1Alpha Pathway36	9.4	CCL22, IL4, IL2, IL1R1, IL1B, IL11
11	Immune System38	9.3	PML, CSF2, CTSD, IFNG, CCR6, IL1R1
12	Selected targets of CREB110	9.3	CRH, INS, CD68, LIF
13	Autoimmune thyroid disease20	9.2	CD40, IL10, IL2, IL4, IFNA1
14	Toll-like receptor signaling pathway20	9.1	TNF, CASP8, IFNA1, FADD, IL1B, CD40
15	Jak-STAT signaling pathway20	9.1	PRL, PTPN6, IFNA1, IL4, IL2, IL11
16	Tec Kinases Signaling36	9.1	CCR7, CCL22, CCR6, FADD, IL4, IL2
17	Rheumatoid arthritis20	9.0	CSF2, CSF1, TNF, IFNG, IL1B, IL17A
18	SUMO Pathway36	8.9	IL1B, IL1R1, TP53, TNF
19	Allograft rejection20	8.9	TNF, IFNG, IL4, IL2, IL10, CD40
20	THC Differentiation Pathway36	8.9	TNF, IFNG, IL4, IL2, IL10
21	Apoptosis20	8.8	CFLAR, IL1B, IL1R1, FADD, CASP8, TP53
22	Endothelin-1 Signaling Pathway36	8.8	CSF1, IL4, IL2, IL1B, IL11, IL10
23	Immune response CD16 signaling in NK cells10	8.8	CSF2, PTPN6, TNF, IFNG, IL4, IL2
24	Malaria20	8.8	PECAM1, TNF, IFNG, IL1B, IL10, CD40
25	Apoptotic Pathways in Synovial Fibroblasts36	8.7	TNF, TP53, CASP8, FADD, IL2, CFLAR
26	ERK Signaling36	8.7	IL4, IL2, IL1B, IL11, IL10, KITLG
27	Cellular Apoptosis Pathway36	8.7	PML, TNF, TP53, CASP8, FADD, IL2
28	Leishmaniasis20	8.6	PTPN6, TNF, IFNG, IL4, IL1B, IL10
29	Molecular Mechanisms of Cancer36	8.6	TP53, CASP8, CCR7, CCL22, CCR6, FADD
30	TGF-Beta Pathway36	8.5	CCL22, IL4, IL2, IL1R1, IL1B, IL11
31	T cell receptor signaling pathway20	8.5	CSF2, PTPN6, TNF, IFNG, IL4, IL2
32	Hematopoietic cell lineage20	8.5	CSF2, KIT, CSF1, TNF, IL4, IL1R1
33	Akt Signaling36	8.4	CSF2, CSF1, TNF, CCR7, CCL22, IL4
34	MIF Mediated Glucocorticoid Regulation36	8.3	CSF2, CSF1, TNF, CCL22, IL4, IL2
35	Tuberculosis20	8.3	CTSD, TNF, CASP8, IFNG, IFNA1, FADD
36	Type I diabetes mellitus20	8.3	TNF, IFNG, IL2, IL1B, INS
37	all-trans-Retinoic Acid Signaling in Brain36	8.3	CCL22, TNF, CSF1, CSF2, IL4, IL2
38	Cytokine Network36	8.3	TNF, IFNG, IFNA1, IL4, IL2, IL1B
39	Chagas disease (American trypanosomiasis)20	8.2	TNF, CASP8, IFNG, FADD, IL2, IL1B
40	MAPK Family Pathway36	8.2	TNF, CCL22, IL4, IL2, IL1R1, IL1B
41	Rho Family GTPases36	8.2	CSF2, CSF1, CCL22, KIT, KITLG, LIF
42	SOCS Pathway36	8.1	CCL22, IL4, IL2, IL1B, IL11, IL10
43	PEDF Induced Signaling36	8.0	CCL22, CASP8, TNF, CSF1, CSF2, IL4
44	STAT3 Pathway36	7.9	CSF2, CSF1, TNF, CCR7, CCL22, CCR6
45	MIF Regulation of Innate Immune Cells36	7.9	TP53, TNF, CSF1, CSF2, CCL22, IL4
46	PAK Pathway36	7.6	CCL22, CASP8, TNF, CSF1, CSF2, IL4
47	JAK-STAT Pathway36	7.5	PRL, IFNG, IFNA1, CCL22, IL4, IL2
48	Antioxidant Action of Vitamin-C36	7.4	CCR6, CCL22, CCR7, TNF, CSF1, CSF2
49	p38 Signaling36	7.0	CCR6, CCL22, CCR7, TP53, TNF, CSF1
50	Cytokine-cytokine receptor interaction20	6.1	IL1R1, IL1B, IL17A, IL11, IL10, FLT3LG

Compounds related to langerhans-cell histiocytosis according to GeneDecks:

(show top 50)    (show all 286)

id	Compound	Score	Top Affiliating Genes
1	cyclophosphamide32 34 9 9	13.7	FLT3LG
2	ivig32	10.5	CSF2, CSF1
3	pge232	9.4	CRH, CTSD, SST, TNFRSF11B, CCL22, IL1RN
4	aspartate32	9.4	CRH, SLC18A2, NOTCH1, OAT, TNFRSF11B, IL11
5	progesterone32 42 9 18 9	13.3	CSF1, PRL, ACTC1, CRH, CTSD, TRH
6	glucose32	9.2	PTPN6, IL11, MKI67, AVP, CFLAR, S100B
7	sb 20358032 42	10.1	CRH, TNFRSF11B, TNF, CASP8, IFNG, IL1R1
8	creatinine32	9.0	CSF1, ACTC1, CRH, CTSD, TNFRSF11B, IL1RN
9	calcitriol32 42 9 18 9	12.8	KITLG, RARA, LIF, CSF2, CSF1, CTSD
10	estrogen32	8.5	INS, MKI67, S100B, S100A1, RARA, IL11
11	paclitaxel32 34 9 9	11.4	CASP8, KIT, ADA, CSF1, KITLG, RARA
12	epinephrine32 9 18 9	11.3	SLC18A2, CSF1, CRH, CASP8, CCL22, IL1RN
13	prostaglandin32	8.2	IL1B, CSF1, KITLG, CRH, TNFRSF11B, TNF
14	doxorubicin32 34 9 9	11.2	CSF1, CTSD, SST, TNFRSF11B, TP53, CASP8
15	etanercept32 9 9	10.1	IL1RN, IL1R1, IL1B, IL17A, IL11, IL10
16	ifn-alpha32	7.9	IL10, IL17A, IL2, GPT, IFNA1, CASP8
17	rantes32	7.9	PECAM1, IL17A, IL11, CD40, LIF, KITLG
18	testosterone32 9 18 9	10.9	MKI67, CFLAR, S100B, LIF, RARA, KITLG
19	gm-csf32	7.8	CSF1, TNF, IFNG, IL4, IL2, IL1B
20	ly29400232	7.8	IL4, RARA, TNFRSF11B, CSF1, IL1B, NOTCH1
21	il-1232	7.8	CSF2, TNF, IFNG, IFNA1, IL4, IL2
22	infliximab32 9 9	9.8	IL1B, IL10, TNFRSF11B, TNF, IFNG, IL4
23	cysteine32	7.7	SLC18A2, PML, CSF2, S100A1, CLEC12A, LIF
24	tgf beta132	7.7	IL1RN, CASP8, TNF, TNFRSF11B, CSF1, KITLG
25	phosphatidylinositol32	7.7	KIT, NCAM1, KITLG, CCR7, FADD, IL1RN
26	paraffin32	7.6	PECAM1, CSF1, ACTC1, CTSD, TP53, F13A1
27	5fluorouracil32	7.5	CTSD, KITLG, KIT, LIF, CFLAR, SST
28	il 1032	7.5	CSF2, CSF1, TNF, IFNG, IL4, IL2
29	cyclosporin a32 42	8.5	PECAM1, ACTC1, CTSD, TNFRSF11B, IFNA1, GPT
30	norepinephrine32 9 18 9	10.4	CRH, INS, SST, S100B, KITLG, LIF
31	fibrinogen32	7.4	CCL22, LIF, F13A1, ALB, CLEC12A, PTPN6
32	histamine32 18	8.3	CCL22, IL4, IL2, IL1RN, IL11, CD40
33	calcium32 9 18 9	10.1	PECAM1, SLC18A2, ACTC1, CTSD, PTPN6, TNFRSF11B
34	prednisolone32 9 9	9.1	TNF, KITLG, ALB, IFNG, IFNA1, GPT
35	indomethacin32 9 9	9.0	ALB, IFNG, IL4, IL2, IL1RN, IL1R1
36	tacrolimus32 34 9 9	10.0	TNF, CASP8, IFNG, CD40, INS, IFNA1
37	neopterin32	6.9	IFNG, IFNA1, GPT, IL4, IL2, IL1RN
38	methotrexate32 34 42 9 9	10.7	IL17A, IL10, MKI67, ITPA, CD40, KITLG
39	vegf32	6.6	TNFRSF11B, S100B, LIF, KITLG, NCAM1, CFLAR
40	actinomycin d32	6.3	IL2, IL1R1, IL1B, IL17A, CD40, CFLAR
41	lactate32	6.3	INS, CTSD, SST, TNF, ALB, MKI67
42	nitric oxide32 9 18 9	8.8	PECAM1, SLC18A2, NOTCH1, CTSD, PTPN6, TNFRSF11B
43	hydrocortisone32 9 9	7.7	CRH, SST, TNFRSF11B, TNF, TRH, IFNG
44	lipid32	5.6	S100B, CLEC12A, LIF, RARA, KITLG, NCAM1
45	thymidine32 18	6.4	CSF1, CTSD, PTPN6, TNF, TP53, TRH
46	tyrosine32	4.8	NOTCH1, IL11, INS, FLT3LG, MKI67, CD40
47	retinoic acid32 42 18	6.8	ADA, PECAM1, SLC18A2, PML, CSF2, CSF1
48	serine32	4.8	NOTCH1, CRH, CTSD, PTPN6, TNFRSF11B, TNF
49	cycloheximide32	4.5	PECAM1, CSF2, CSF1, CRH, CTSD, SST
50	dexamethasone32 42 34 9 9	7.8	IFNA1, CD163, AVP, S100B, CLEC12A, LIF

Cellular components related to langerhans-cell histiocytosis according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	death-inducing signaling complex	GO:031264	9.7	CASP8, FADD, CFLAR
2	external side of plasma membrane	GO:009897	8.3	ADA, TNF, IFNG, CCR7, IL4, IL17A
3	plasma membrane	GO:005886	7.3	IL1RN, CCR6, CCR7, TRH, TNF, NOTCH1
4	extracellular region	GO:005576	5.2	TNFRSF11B, TRH, F13A1, ALB, SST, CTSD
5	extracellular space	GO:005615	4.0	KIT, IFNG, ALB, TNF, TNFRSF11B, SST

Biological processes related to langerhans-cell histiocytosis according to GeneDecks:

(show all 42)

id	Name	GO ID	Score	Top Affiliating Genes
1	extrinsic apoptotic signaling pathway	GO:097191	10.1	FADD, CASP8, TNF
2	positive regulation of peptidyl-serine phosphorylation	GO:033138	10.1	TNF, IL11, AVP, LIF
3	response to ethanol	GO:045471	10.1	ACTC1, TRH, CASP8, IL4, AVP
4	positive regulation of interleukin-23 production	GO:032747	10.1	CSF2, IFNG, IL17A
5	positive regulation of peptidyl-serine phosphorylation of STAT protein	GO:033141	10.1	IFNG, LIF
6	chronic inflammatory response to antigenic stimulus	GO:002439	10.1	IL1RN, IL1B
7	positive regulation of JAK-STAT cascade	GO:046427	10.0	KIT, NOTCH1, PRL
8	positive regulation of tyrosine phosphorylation of Stat1 protein	GO:042511	10.0	IFNG, CD40, LIF, KIT
9	positive regulation of B cell proliferation	GO:030890	10.0	ADA, IL4, IL2, CD40
10	negative regulation of glutamate secretion	GO:014050	9.9	IL1B, IL1RN, TRH
11	positive regulation of osteoclast differentiation	GO:045672	9.9	CSF1, TNF, IFNG, IL17A
12	embryonic hemopoiesis	GO:035162	9.9	KIT, KITLG, FLT3LG
13	regulation of isotype switching	GO:045191	9.9	IL4, IL10
14	positive regulation of isotype switching to IgG isotypes	GO:048304	9.8	IFNG, IL4, IL2, CD40
15	positive regulation of vitamin D biosynthetic process	GO:060557	9.8	TNF, IFNG
16	cellular response to mechanical stimulus	GO:071260	9.7	CD40, IL1B, FADD, CASP8
17	positive regulation of tyrosine phosphorylation of Stat5 protein	GO:042523	9.7	CSF2, IL4, IL2, KIT
18	positive regulation of MHC class II biosynthetic process	GO:045348	9.7	IL10, IL4, IFNG
19	hemopoiesis	GO:030097	9.6	KIT, KITLG, IL10, CSF1
20	positive regulation of calcidiol 1-monooxygenase activity	GO:060559	9.6	TNF, IFNG, IL1B
21	defense response to virus	GO:051607	9.5	IFNG, FADD, CD40, CD207
22	elevation of cytosolic calcium ion concentration	GO:007204	9.5	CCR7, CCR6, IL2, IL1B, AVP
23	cell proliferation	GO:008283	9.4	CSF1, PRL, PTPN6, TP53, MKI67, FSCN1
24	positive regulation of membrane protein ectodomain proteolysis	GO:051044	9.4	IL1B, IFNG, TNF
25	negative regulation of growth of symbiont in host	GO:044130	9.4	TNF, IFNG, IL10
26	positive regulation of chemokine biosynthetic process	GO:045080	9.4	IL1B, IL4, IFNG, TNF
27	positive regulation of I-kappaB kinase/NF-kappaB cascade	GO:043123	9.3	TNF, CASP8, CCR7, FADD, IL1B, CD40
28	positive regulation of peptidyl-tyrosine phosphorylation	GO:050731	9.2	TP53, IL11, INS, LIF, KITLG
29	positive regulation of MAPK cascade	GO:043410	9.2	IL11, INS, LIF, KIT
30	negative regulation of lipid catabolic process	GO:050995	9.0	TNF, IL1B, INS
31	positive regulation of sequence-specific DNA binding transcription factor activity	GO:051091	8.9	TNF, IL4, IL1B, IL10, KIT
32	immune response	GO:006955	8.8	CCR6, CCL22, CCR7, NOTCH1, CSF2, IL4
33	positive regulation of nitric oxide biosynthetic process	GO:045429	8.7	TNF, IFNG, IL1B, INS
34	cytokine-mediated signaling pathway	GO:019221	8.7	PML, PTPN6, IFNG, IL1B, KIT, NCAM1
35	signal transduction	GO:007165	8.6	PECAM1, CRH, TNFRSF11B, TRH, CCL22, CCR6
36	response to drug	GO:042493	8.6	ACTC1, SST, TNFRSF11B, TNF, IFNG, IL4
37	inflammatory response	GO:006954	8.5	CCR7, TNF, CRH, CSF1, CCL22, IL1B
38	positive regulation of cell proliferation	GO:008284	8.3	KIT, CSF2, CSF1, NOTCH1, PTPN6, IL2
39	apoptotic process	GO:006915	8.1	TNF, TNFRSF11B, PTPN6, ACTC1, TP53, CASP8
40	cell-cell signaling	GO:007267	7.9	SST, TRH, CCL22, IL2, IL1B, IL17A
41	positive regulation of transcription from RNA polymerase II promoter	GO:045944	7.9	FADD, IFNG, TP53, TNF, NOTCH1, IL4
42	negative regulation of apoptotic process	GO:043066	7.7	CSF2, TNF, TP53, ALB, IL4, IL1RN

Molecular functions related to langerhans-cell histiocytosis according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	growth factor activity	GO:008083	8.1	CSF2, CSF1, IL4, IL2, IL1B, IL11
2	protease binding	GO:002020	8.0	TNF, TP53, IL1R1, INS, KIT
3	cytokine activity	GO:005125	7.0	KITLG, CSF2, CSF1, TNFRSF11B, TNF, IFNG
4	protein binding	GO:005515	5.0	FADD, ALB, CASP8, TP53, TNF, PTPN6