Histiocytosis disease: Malacards - Research Articles, Drugs, Genes, Clinical Trials

MCID: HST010 MIFTS: 49	Histiocytosis Categories: Immune diseases	Data Licensing For inquiries, contact: [email protected]
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Aliases & Classifications for Histiocytosis

MalaCards integrated aliases for Histiocytosis:

Name: Histiocytosis ¹¹ ⁵³ ⁴³ ¹⁴ ⁷¹ ⁷⁵

Hand Schuller Christian Disease ¹¹

Hand-Schüller-Christian Disease ⁷⁵

Histiocytosis, Langerhans-Cell ⁷¹

Chronic Histiocytosis X ¹¹

Histiocytic Syndrome ⁷¹

Classifications:

MalaCards categories:
Anatomical: Immune diseases

See all MalaCards categories (disease lists)

External Ids:

Disease Ontology ¹¹ DOID:3405

MeSH ⁴³ D015614

NCIt ⁴⁹ C3106

SNOMED-CT ⁶⁸ 60657004

UMLS ⁷¹ C0019618 C0019621 C0398597

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Summaries for Histiocytosis

Disease Ontology: ¹¹ A lymphatic system disease that is characterized by an excessive number of histiocytes.

MalaCards based summary: Histiocytosis, also known as hand schuller christian disease, is related to histiocytosis-lymphadenopathy plus syndrome and langerhans cell sarcoma. An important gene associated with Histiocytosis is SLC29A3 (Solute Carrier Family 29 Member 3), and among its related pathways/superpathways are Innate Immune System and MIF Mediated Glucocorticoid Regulation. The drugs Prednisone and Mycophenolic acid have been mentioned in the context of this disorder. Affiliated tissues include lung, bone and liver, and related phenotypes are Misorientated spindle and homeostasis/metabolism

Wikipedia ⁷⁵ Histiocytosis: In medicine, histiocytosis is an excessive number of histiocytes (tissue macrophages), and the term is... more...

Hand-schüller-christian disease: Chronic multifocal Langerhans cell histiocytosis, previously known as Hand-Schüller-Christian disease,... more...

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Related Diseases for Histiocytosis

Diseases related to Histiocytosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 1243)

#	Related Disease	Score	Top Affiliating Genes
1	histiocytosis-lymphadenopathy plus syndrome	32.4	SLC29A3 SERPINA3 S100B CD1C CD1A CD163
2	langerhans cell sarcoma	31.9	CD207 CD1C CD1A
3	fibrosarcoma of bone	31.8	TNFRSF8 SERPINA3 CD207 CD1C CD1A
4	histiocytic sarcoma	31.6	SERPINA3 CD1C CD1A CD163 BRAF
5	juvenile xanthogranuloma	31.6	SERPINA3 CD207 CD1C CD1A CD163
6	erdheim-chester disease	31.5	SERPINA3 NRAS MAP2K1 CD1C CD1A CCR6
7	multicentric reticulohistiocytosis	31.5	SERPINA3 CD1C CD1A
8	reticulohistiocytic granuloma	31.5	SERPINA3 CD207 CD1C CD1A CD163
9	exanthem	31.2	TNF CCR6 ALK
10	interdigitating dendritic cell sarcoma	31.1	SERPINA3 CD207 CD1C CD1A CD163
11	lymphoproliferative syndrome	30.7	TNFRSF8 NRAS CCR6 ALK
12	otorrhea	30.7	CD207 CD1C CD1A
13	lymphoma, hodgkin, classic	30.7	TNFRSF8 PTPRC CCR6 ALK
14	non-langerhans-cell histiocytosis	30.6	U2AF1 SERPINA3 NRAS MAP2K1 CD207 CD1C
15	sarcoidosis 1	30.5	TNF SERPINA3 PTPRC CCR6
16	hemophagocytic lymphohistiocytosis	30.5	U2AF1 TNF CD163 CCR6
17	lymphadenitis	30.5	TNF SERPINA3 CCR6
18	reticulosarcoma	30.4	TNFRSF8 SERPINA3 PTPRC ALK
19	marginal zone b-cell lymphoma	30.4	U2AF1 TNFRSF8 CCR6
20	folliculitis	30.2	TNF CD1A CCR6
21	differentiated thyroid carcinoma	30.2	NRAS BRAF ALK
22	seborrheic dermatitis	30.2	TNF CD207 CD1C CD1A CCR6
23	lethal midline granuloma	30.2	TNFRSF8 SERPINA3
24	langerhans cell histiocytosis	30.1	U2AF1 TNFRSF8 TNF SERPINA3 S100B NRAS
25	fibrous histiocytoma	30.0	SERPINA3 S100B CD163 ALK
26	lymphatic system disease	30.0	U2AF1 TNFRSF8 SERPINA3 CCR6
27	kimura disease	30.0	TNFRSF8 PECAM1 CD1A
28	spinal cord disease	30.0	TNF SERPINA3 CCR6
29	anterior uveitis	30.0	TNF SERPINA3 CCR6
30	interstitial lung disease 2	30.0	TNF SERPINA3 PTPRC PECAM1 CCR6
31	peripheral t-cell lymphoma	30.0	TNFRSF8 PTPRC CCR6 ALK
32	lymph node disease	30.0	TNF SERPINA3 CCR6
33	orbital cancer	30.0	CD1C CD1A
34	myeloma, multiple	30.0	U2AF1 TNFRSF8 PTPRC NRAS CCR6 BRAF
35	monoclonal gammopathy of uncertain significance	29.9	U2AF1 PTPRC CCR6
36	celiac disease 1	29.9	TNFRSF8 TNF SERPINA3 PTPRC CCR6
37	plasma cell neoplasm	29.9	U2AF1 PTPRC NRAS BRAF
38	angioimmunoblastic t-cell lymphoma	29.9	TNFRSF8 CCR6 ALK
39	castleman disease	29.9	TNFRSF8 S100B CCR6
40	granuloma annulare	29.9	TNF CD1C CD1A CD163
41	hematologic cancer	29.9	U2AF1 TNFRSF8 SERPINA3 CCR6 ALK
42	pleural disease	29.9	TNF CCR6 ALK
43	mycosis fungoides	29.9	TNFRSF8 CD1C CD1A CCR6 ALK
44	melanoma in congenital melanocytic nevus	29.9	S100B NRAS BRAF
45	hairy cell leukemia	29.8	U2AF1 PTPRC BRAF
46	malignant fibrous histiocytoma	29.8	SERPINA3 S100B PTPRC
47	necrobiosis lipoidica	29.8	TNF CD1A CD163
48	severe combined immunodeficiency	29.8	TNFRSF8 TNF PTPRC PECAM1 CCR6
49	ewing sarcoma	29.8	TNFRSF8 TNF S100B PTPRC ALK
50	central nervous system vasculitis	29.8	TNF SERPINA3 CCR6

Graphical network of the top 20 diseases related to Histiocytosis:

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Symptoms & Phenotypes for Histiocytosis

GenomeRNAi Phenotypes related to Histiocytosis according to GeneCards Suite gene sharing:

²⁵

#	Description	GenomeRNAi Source Accession	Score	Top Affiliating Genes
1	no effect	GR00402-S-1	10.15	ALK ARAF BRAF CCR6 CD101 CD163
2	no effect	GR00402-S-2	10.15	ALK ARAF BRAF CCR6 CD101 CD1A
3	Misorientated spindle	GR00256-A	9.13	ALK ARAF BRAF

MGI Mouse Phenotypes related to Histiocytosis:

⁴⁵

#	Description	MGI Source Accession	Score	Top Affiliating Genes
1	homeostasis/metabolism	MP:0005376	10.17	ALK BRAF CCR6 CD163 MAP2K1 NRAS
2	immune system	MP:0005387	10.1	ARAF BRAF CCR6 CD101 CD163 CD207
3	neoplasm	MP:0002006	9.97	ALK BRAF MAP2K1 NRAS PTPRC SLC29A3
4	hematopoietic system	MP:0005397	9.8	ARAF BRAF CCR6 CD101 CD163 CD207
5	respiratory system	MP:0005388	9.76	ALK BRAF CD163 CD207 PECAM1 PTPRC
6	integument	MP:0010771	9.28	ALK BRAF MAP2K1 NRAS PTPRC S100B

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Drugs & Therapeutics for Histiocytosis

Drugs for Histiocytosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 134)

Name

Status

Phase

Clinical Trials

Cas Number

PubChem Id

Prednisone

Approved, Vet_approved

Phase 4

53-03-2

5865

Mycophenolic acid

Approved, Investigational

Phase 4

24280-93-1

446541

Antineoplastic Agents, Hormonal

Phase 4

Anti-Bacterial Agents

Phase 4

Anti-Infective Agents

Phase 4

Hormones

Phase 4

Hormone Antagonists

Phase 4

glucocorticoids

Phase 4

Antitubercular Agents

Phase 4

Anti-Inflammatory Agents

Phase 4

Antibiotics, Antitubercular

Phase 4

Vinblastine

Approved

Phase 3

865-21-4

241903 13342

Prednisolone phosphate

Approved, Vet_approved

Phase 3

302-25-0

Prednisolone acetate

Approved, Vet_approved

Phase 3

52-21-1

Prednisolone

Approved, Vet_approved

Phase 3

50-24-8

4894 5755

Mercaptopurine

Approved

Phase 3

50-44-2

667490

Methylprednisolone hemisuccinate

Approved

Phase 3

2921-57-5

1875

Methylprednisolone

Approved, Vet_approved

Phase 3

83-43-2

4159 6741

Cytarabine

Approved, Investigational

Phase 3

147-94-4

6253

Levoleucovorin

Approved, Experimental, Investigational

Phase 2, Phase 3

68538-85-2, 58-05-9, 73951-54-9

149436 6006

Methotrexate

Approved

Phase 2, Phase 3

1959-05-2, 59-05-2

4112 126941

Vincristine

Approved, Investigational

Phase 3

2068-78-2, 57-22-7

5978

Cladribine

Approved, Investigational

Phase 2, Phase 3

4291-63-8

20279

Indomethacin

Approved, Investigational

Phase 2, Phase 3

53-86-1

3715

Cyclophosphamide

Approved, Investigational

Phase 2, Phase 3

50-18-0, 6055-19-2

2907

Busulfan

Approved, Investigational

Phase 2, Phase 3

55-98-1

2478

Folic acid

Approved, Nutraceutical, Vet_approved

Phase 2, Phase 3

59-30-3

6037

Prednisolone hemisuccinate

Experimental

Phase 3

2920-86-7

4897

Vitamins

Phase 3

Trace Elements

Phase 3

Antidotes

Phase 3

Micronutrients

Phase 3

Liver Extracts

Phase 3

Antiviral Agents

Phase 3

Antimitotic Agents

Phase 3

Tubulin Modulators

Phase 3

Methylprednisolone Acetate

Phase 3

584547

Antirheumatic Agents

Phase 2, Phase 3

Folic Acid Antagonists

Phase 2, Phase 3

Folate

Phase 2, Phase 3

Vitamin B9

Phase 2, Phase 3

Vitamin B Complex

Phase 2, Phase 3

Dermatologic Agents

Phase 2, Phase 3

Immunoglobulins, Intravenous

Phase 2, Phase 3

Immunoglobulins

Phase 2, Phase 3

Antibodies

Phase 2, Phase 3

gamma-Globulins

Phase 2, Phase 3

Rho(D) Immune Globulin

Phase 2, Phase 3

Anti-Inflammatory Agents, Non-Steroidal

Phase 2, Phase 3

Analgesics, Non-Narcotic

Phase 2, Phase 3

Interventional clinical trials:

(show top 50) (show all 75)

#	Name	Status	NCT ID	Phase	Drugs
1	A Trial to Evaluate the Long Term Prognosis in Rosai-Dorfman Disease	Recruiting	NCT05284942	Phase 4	Mycophenolate mofetil combined with oral prednisone
2	H-9926-LCH III: Treatment Protocol of the Third International Study for Langerhans Cell Histiocytosis	Completed	NCT00488605	Phase 3	Prednisone, Vinblastin, 6-mercaptopuroine;Leucovorin, Methotrexate, Vinblastine, Prednisone
3	Randomization of Cytarabine Monotherapy Versus Standard-of-Care Vinblastine/Prednisone for Frontline Treatment of Langerhans Cell Histiocytosis (TXCH LCH0115)	Recruiting	NCT02670707	Phase 3	Cytarabine;Vinblastine/prednisone
4	A Prospective Institutional Study for the Treatment of Children With Newly Diagnosed Langerhans Cell Histiocytosis Using a Cytarabine Contained Protocol	Recruiting	NCT04773366	Phase 3	Prednisone+Cytarabine+vincristine;Prednisone+Cytarabine+vincristine+Mercaptopurine;Prednisone+vincristine+Mercaptopurine;Prednisone+vincristine
5	LCH-IV, International Collaborative Treatment Protocol for Children and Adolescents With Langerhans Cell Histiocytosis	Recruiting	NCT02205762	Phase 2, Phase 3	Prednisone;Vinblastine;mercaptopurine;INDOMETHACIN;Methotrexate;Cytosine Arabinoside;2-chlorodeoxyadenosine
6	Determination of Molecular Status, as Well as the Efficacy and Safety of Fluorodeoxyglucose (18F-FDG) in PET-CT Imaging in Juvenile Patients With Histiocytosis	Recruiting	NCT04943211	Phase 3	fluorodeoxyglucose (18F-FDG)
7	Cobimetinib for BRAF-wild-type or Mutated Histiocytoses : a Randomized, Placebo-controlled, Double Blind Study" COBRAH Study	Active, not recruiting	NCT04007848	Phase 3	Cobimetinib;Placebo oral tablet
8	In-vivo T-cell Depletion and Hematopoietic Stem Cell Transplantation for Life-Threatening Immune Deficiencies and Histiocytic Disorders	Terminated	NCT00176826	Phase 2, Phase 3	Myeloablative conditioning regimen
9	The Use Of Umbilical Cord Blood As A Source Of Hematopoietic Stem Cells	Unknown status	NCT00084695	Phase 2	busulfan;cyclophosphamide;fludarabine phosphate;melphalan;methylprednisolone
10	The Prospective Non-randomized Phase II Clinical Trial of Vemurafenib in Combination With Cytarabine and 2-chlorodeoxyadenosine in Children With Langerhans-cell Hisitocytosis With BRAF V600E Mutation	Unknown status	NCT03585686	Phase 2	Vemurafenib;Cytarabine;2-chlorodeoxyadenosine
11	Transplantation Using Umbilical Cord And Placental Blood	Unknown status	NCT00008164	Phase 2
12	Cord Blood Ex-Vivo MSC Expansion Plus Fucosylation to Enhance Homing and Engraftment	Completed	NCT03096782	Phase 2	Busulfan;Clofarabine;Cyclophosphamide;Fludarabine;Melphalan;Mycophenolate Mofetil;Tacrolimus
13	Evaluation of Efficacy of Denosumab in Adult Patients With Langerhans Cell Histiocytosis (LCH): a Multiple-site, Single Arm, Open Label Clinical Trial	Completed	NCT03270020	Phase 2	Denosumab 70 MG/ML [Xgeva]
14	Efficacy and Tolerance of Combination Chemotherapy With Methotrexate and Cytosine Arabinoside in Newly Diagnosed Adult With Langerhans Cell Histiocytosis	Completed	NCT02389400	Phase 2	methotrexate
15	Allogeneic Hematopoietic Stem Cell Transplant for Patients With Immunologic or Histiocytic Disorders Using a Non-Myeloablative Preparative Regimen to Achieve Stable Mixed Chimerism	Completed	NCT00176865	Phase 2	Fludarabine;Melphalan;Anti-thymocyte globulin (ATG);Campath 1H;Cyclosporin A;Mycophenolate mofetil;Intravenous immunoglobulin (IVIG)
16	Treatment of Resistant Langerhans Cell Histiocytosis With Etanercept (ENBREL, IMMUNEX, SEATTLE)	Completed	NCT00048373	Phase 2	Etanercept
17	Study of Sequential Administration of Oral 6-Thioguanine After Methotrexate in Patients With Langerhans Cell in Histiocytosis (LCH)	Completed	NCT00588536	Phase 2	Methotrexate;6-Thioguanine;Leucovorin Calcium
18	A Phase 2a, Open Label, Multicenter Study to Assess the Efficacy and Safety of the Oral AKT Inhibitor GSK2110183 in Subjects With Langerhans Cell Histiocytosis	Completed	NCT01395004	Phase 2	GSK2110183
19	Impact of Intervention With Deferasirox on the Immune Function of Patients With Hematologic Diseases and Transfusion-Related Iron Overload	Completed	NCT01273766	Phase 2	deferasirox
20	HLA-Nonidentical Stem Cell and Natural Killer Cell Transplantation for Children Less the Two Years of Age With Hematologic Malignancies	Completed	NCT00145626	Phase 2	Chemotherapy and antibodies
21	CD34+Stem Cell Selection for Patients Receiving Partially Matched Family or Matched Unrelated Adult Donor Allogeneic Stem Cell Transplantations for Malignant and Non-Malignant Disease	Completed	NCT01049854	Phase 2	Full Intensity with TBI;Full Intensity;Reduced Intensity;Reduced Intensity (Fanconi)
22	NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) Screening Protocol	Recruiting	NCT03155620	Phase 2	Ensartinib;Erdafitinib;Larotrectinib Sulfate;Olaparib;Palbociclib;Samotolisib;Selpercatinib;Selumetinib Sulfate;Tazemetostat;Tipifarnib;Ulixertinib;Vemurafenib
23	A Phase 2 Study to Assess the Safety and Efficacy of Cobimetinib in Refractory Langerhans Cell Histiocytosis, LCH-Associated Neurodegenerative Disease, and Other Histiocytic Disorders.	Recruiting	NCT04079179	Phase 2	Cobimetinib
24	NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice)- Phase 2 Subprotocol of Tipifarnib in Patients With Tumors Harboring HRAS Genomic Alterations	Recruiting	NCT04284774	Phase 2	Tipifarnib
25	NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of AG-120 (Ivosidenib) in Patients With Tumors Harboring IDH1 Mutations	Recruiting	NCT04195555	Phase 2	Ivosidenib
26	NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of LOXO-292 in Patients With Tumors Harboring RET Gene Alterations	Recruiting	NCT04320888	Phase 2	Selpercatinib
27	NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of LOXO-101 (Larotrectinib) in Patients With Tumors Harboring Actionable NTRK Fusions	Recruiting	NCT03213704	Phase 2	Larotrectinib Sulfate
28	NCI-COG Pediatric MATCH (Molecular Analysis For Therapy Choice)- Phase 2 Subprotocol of LY3023414 in Patients With Solid Tumors	Recruiting	NCT03213678	Phase 2	Samotolisib
29	Thalidomide, Cyclophosphamide and Dexamethasone for Adult Patients With Recurrent/Refractory Langerhans Cell Histiocytosis: A Single Arm, Single Center, Prospective Phase 2 Study	Recruiting	NCT04120519	Phase 2	thalidomide combined with dexamethasone and cyclophosphamide
30	Cytarabine Monotherapy for Adult Patients With Newly Diagnosed Langerhans Cell Histiocytosis: A Single Arm, Single Center, Prospective Phase 2 Study	Recruiting	NCT04121819	Phase 2	Cytarabine
31	A Single-arm, Open Label, Multicenter Phase II Clinical Study in Rare Diseases to Evaluate Safety, Efficacy and PK of HLX208 for Adult Langerhans Cell Histiocytosis (LCH) and Erdheim-Chester Disease (ECD) With BRAF V600E Mutation	Recruiting	NCT05092815	Phase 2	HLX208
32	Optimization of the Time and Dosage of Trametinib in BRAF Negative Juvenile Patients With Refractory Histiocytosis or After the Failure of Vemurafenib Treatment	Recruiting	NCT04943224	Phase 2	Trametinib
33	Optimization of the Time and Dosage of Vemurafenib in BRAF Positive Juvenile Patients With Refractory Histiocytosis	Recruiting	NCT04943198	Phase 2	Vemurafenib
34	Lenalidomide and Dexamethasone for Rosai-Dorfman Disease: A Single Arm, Single Center, Prospective Phase 2 Study	Recruiting	NCT04924647	Phase 2	lenalidomide and dexamethasone
35	NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of BVD-523FB (Ulixertinib) in Patients With Tumors Harboring Activating MAPK Pathway Mutations	Active, not recruiting	NCT03698994	Phase 2	Ulixertinib
36	NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of Palbociclib in Patients With Tumors Harboring Activating Alterations in Cell Cycle Genes	Active, not recruiting	NCT03526250	Phase 2	Palbociclib
37	NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice)- A Phase 2 Subprotocol of Olaparib in Patients With Tumors Harboring Defects in DNA Damage Repair Genes	Active, not recruiting	NCT03233204	Phase 2	Olaparib
38	NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of Tazemetostat in Patients With Tumors Harboring Alterations in EZH2 or Members of the SWI/SNF Complex	Active, not recruiting	NCT03213665	Phase 2	Tazemetostat
39	CD34+ Stem Cell Selection for Patients Receiving a Matched or Partially Matched Family or Unrelated Adult Donor Allogeneic Stem Cell Transplantation for Non-Malignant Disease	Active, not recruiting	NCT01966367	Phase 1, Phase 2
40	Phase II Study of Clofarabine in Patients With Recurrent or Refractory Langerhans Cell Histiocytosis and LCH-related Disorders	Active, not recruiting	NCT02425904	Phase 2	Clofarabine
41	A Phase II Study of Lenalidomide for Adult Histiocyte Disorders	Active, not recruiting	NCT02523040	Phase 2	Lenalidomide
42	NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice)- Phase 2 Subprotocol of Vemurafenib in Patients With Tumors Harboring BRAF V600 Mutations	Active, not recruiting	NCT03220035	Phase 2	Vemurafenib
43	Evaluation of Efficacy and Tolerance of Cladribine in Symptomatic Patients With Pulmonary Langerhans Cell Histiocytosis and Impairment of Lung Function	Active, not recruiting	NCT01473797	Phase 2	Cladribine
44	Phase 2 Study of DAY101 in Relapsed and Refractory Langerhans Cell Histiocytosis	Not yet recruiting	NCT05287295	Phase 2	Tovorafenib
45	NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice)- Phase 2 Subprotocol of Ensartinib in Patients With Tumors Harboring ALK or ROS1 Genomic Alterations	Suspended	NCT03213652	Phase 2	Ensartinib
46	NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of Erdafitinib in Patients With Tumors Harboring FGFR1/2/3/4 Alterations	Suspended	NCT03210714	Phase 2	Erdafitinib
47	Reduced Intensity Hematopoietic Cell Transplantation for Patients With Resistant Langerhans Cell Histiocytosis	Terminated	NCT00618540	Phase 2	fludarabine phosphate;melphalan
48	Unrelated Donor Stem Cell Transplant for Patients With Malignant and Non-Malignant Disorders	Terminated	NCT01050439	Phase 2
49	Phase II Study of Clofarabine in Patients With Recurrent or Refractory Langerhans Cell Histiocytosis	Withdrawn	NCT01796405	Phase 2	Clofarabine
50	A Phase 1/Pharmacokinetic Study of Sunitinib in Patients With Cancer Who Also Have HIV and Are on HAART Therapy	Completed	NCT00890747	Phase 1	sunitinib malate

Search NIH Clinical Center for Histiocytosis

Inferred drug relations via UMLS ⁷¹ / NDF-RT ⁵⁰ :

Etoposide

etoposide phosphate

Thalidomide

Vinblastine

Vinblastine Sulfate

Cochrane evidence based reviews: histiocytosis

Sources

Genetic Tests for Histiocytosis

Sources

Anatomical Context for Histiocytosis

Organs/tissues related to Histiocytosis:

MalaCards : Lung, Bone, Liver, Lymph Node, Bone Marrow, Pituitary, Thyroid

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Publications for Histiocytosis

Articles related to Histiocytosis:

(show top 50) (show all 9837)

#	Title	Authors	PMID	Year
1	[Eosinophilic granuloma of the temporal bone. Case report and literature review]. ⁵³ ⁶²	Hellmann M...Sudhoff H	12717600	2003
2	Oral submucosal dendrocytes: factor XIIIa+ and CD34+ dendritic cell populations in normal tissue and fibrovascular lesions. ⁵³ ⁶²	Regezi JA...Headington JT	1282135	1992
3	A case of renal involvement in juvenile xanthogranulomatosis. ⁶²	Shibuki S...Nakajima T	36439921	2023
4	Rosai-Dorfman disease presenting as stridor and hoarseness in a young female patient. ⁶²	Kokje V...Mermod M	36452890	2023
5	Outcomes of liver transplantation in children with Langerhans cell histiocytosis: Experience from a quaternary care center. ⁶²	Menon J...Rela M	36317422	2023
6	The added value of skeletal surveys in the initial evaluation of children diagnosed with Langerhans cell histiocytosis in the era of staging 18 F-FDG PET/CT: A retrospective study. ⁶²	Rameh V...Tsai A	36266951	2023
7	PET/CT Showing a Case of Langerhans Cell Histiocytosis Involving the Pleura. ⁶²	Tang P...Tian R	36469071	2023
8	Histiocytic Diseases. ⁶²	Moore PF	36270835	2023
9	Langerhans Cell Histiocytosis Appearing as Wandering Pulmonary Nodules. ⁶²	Manabe T...Yatera K	35342142	2022
10	Optimal timing of liver transplantation for liver cirrhosis caused by sclerosing cholangitis in a patient with Langerhans cell histiocytosis: a case report. ⁶²	Watakabe M...Koh K	36469185	2022
11	Preceding polydipsia/polyuria, ataxia, and dysarthria in an adult with mixed histiocytosis (Erdheim Chester disease/Langerhans cell histiocytosis). ⁶²	Miyoshi T...Imashuku S	36197468	2022
12	Kingella kingae Osteomyelitis Should be Ruled out in Pediatric Langerhans Cell Histiocytosis. ⁶²	Yagupsky P	36102720	2022
13	Classic cytomorphological features of Langerhans cell histiocytosis with osseous involvement. ⁶²	Rolon MDMR...Khazai L	36047490	2022
14	Diagnosis of Langerhans cell histiocytosis on cytological examination of cerebrospinal fluid: Report of the first case. ⁶²	Tommasino F...Caputo A	35950687	2022
15	Paediatric Strategy Forum for medicinal product development in mitogen-activated protein kinase pathway inhibitors: ACCELERATE in collaboration with the European Medicines Agency with participation of the Food and Drug Administration. ⁶²	Pearson AD...Vassal G	36335782	2022
16	Benign cephalic histiocytosis with extra-facial manifestations. ⁶²	Bertino L...Vaccaro M	35950714	2022
17	Letter to the Editor Regarding "Local ALK-Positive Histiocytosis with Unusual Morphology and Novel TRIM33::ALK Gene Fusion" by Tran et al. ⁶²	Soylemez Akkurt T...Baskan F	36471533	2022
18	Crystal-storing histiocytosis associated with monoclonal kappa light chain gammopathy. ⁶²	Lesesve JF...Thomas B	35451182	2022
19	Strong Coexpression of Transcription Factors PU.1 and Oct-2 in Rosai-Dorfman Disease. ⁶²	Kiruthiga KG...Natkunam Y	36239684	2022
20	68Ga-FAPI and 18F-FDG PET/CT Images of a Patient With Rosai-Dorfman Disease With Liver Involvement. ⁶²	Chen Z...Miao W	36342795	2022
21	A Review of Noninfectious Diseases Masquerading as Acute Mastoiditis. ⁶²	Wong K...Perez ER	34874762	2022
22	Incidence, prevalence and survival in patients with Langerhans cell histiocytosis: A national registry study from England, 2013-2019. ⁶²	Liu H...West J	36122574	2022
23	The international dataset on the association between Langerhans Cell Histiocytosis and other malignancies. ⁶²	Bagnasco F...Haupt R	36188132	2022
24	Biomodulatory therapy induces durable remissions in multi-system Langerhans cell histiocytosis. ⁶²	Harrer DC...Heudobler D	35819881	2022
25	High-Resolution Computed Tomography of Cystic Lung Disease. ⁶²	Kusmirek JE...Meyer CA	36252611	2022
26	Narrowing the Differential Diagnosis of Cystic Lesions in Smokers with Expiratory CT Acquisition Using the Cyst-Airway Communication Hypothesis. ⁶²	Hochhegger B...Marchiori E	36271930	2022
27	Response to: Langerhans Cell Histiocytosis Associated With Renal Cell Carcinoma: Is it an Indolent Phenomenon or a More Ominous Pathologic Process? ⁶²	Agaimy A	36156506	2022
28	Langerhans Cell Histiocytosis Associated With Renal Cell Carcinoma: Is it an Indolent Phenomenon or a More Ominous Pathologic Process? ⁶²	Tran TAN...Mekhail T	35838640	2022
29	Multicentric Reticulohistiocytosis Associated with an Early Form of Systemic Lupus Erythematosus: A Case Report of a Rare Disease, with Mini Review of the Literature. ⁶²	Mariotti EB...Caproni M	36362761	2022
30	Erdheim-Chester disease: look it in the eye. An orbital magnetic resonance imaging study. ⁶²	Haroche J...Shor N	35484665	2022
31	Airspace Enlargement with Fibrosis in a Young Heavy Smoker Mimicking Diffuse Cystic Lung Disease. ⁶²	Bae K...Jeon KN	36422188	2022
32	Unexplained fever in children-Benefits and challenges of FDG-PET/CT. ⁶²	Nygaard U...Borgwardt L	36210785	2022
33	Imaging of Smoking and Vaping Related Diffuse Lung Injury. ⁶²	Cheng KA...Washington L	36202480	2022
34	Solitary Involvement of the Liver: A Rare Manifestation of Langerhans Cell Histiocytosis. ⁶²	Consing M...Vahidi S	36404611	2022
35	Rosai-Dorfman-Destombes Disease in the Pediatric Head and Neck. ⁶²	Lee NK...Herrmann BW	36330598	2022
36	MUM1/IRF4 is Highly Expressed in Dermatopathic Lymphadenopathy: Potential Utility in Diagnosis and Differential Diagnosis. ⁶²	Garces S...Medeiros LJ	35877199	2022
37	[Rosai-Dorfman-Destombes disease with a misleading laryngeal presentation]. ⁶²	Fiani DJ...Fontaine J	36038429	2022
38	Eye movement abnormalities in neurodegenerative langerhans cell histiocytosis. ⁶²	Autier L...Idbaih A	35760933	2022
39	Immune-mediated diseases involving central and peripheral nervous systems. ⁶²	Leboyan A...Taieb G	36366904	2022
40	Clinical response to dabrafenib and chemotherapy in clonally-related histiocytosis and acute lymphoblastic leukemia. ⁶²	Hubert G...Tran TH	36384252	2022
41	Curative Pericardiectomy in Interpheron-Resistant Severe Pericardial Erdheim-Chester Disease. ⁶²	Gomez-Herrero J...Fuentes RG	36444187	2022
42	Congenital solitary reticulohistiocytosis (Hashimoto - Pritzker). ⁶²	Lima LPN...Souza EM	36153176	2022
43	Rare presentation of multiple thromboses in Erdheim-Chester disease: a case-based review. ⁶²	Liu Y...Wang M	35687294	2022
44	Does Fractal Analysis Have a Role in Diagnosis of Langerhans Cell Histiocytosis? ⁶²	Sinanoglu A...Ozcan I	35988692	2022
45	BRAF-V600E utilizes posttranscriptional mechanisms to amplify LPS-induced TNFα production in dendritic cells in a mouse model of Langerhans cell histiocytosis. ⁶²	Minichino D...Behrens EM	35648675	2022
46	A rare case of Haemophagocytic Lymphohistiocytosis preceding a diagnosis of Langerhans cell histiocytosis. ⁶²	Riley G...Pettit T	35652532	2022
47	Open and Endoscopic Endonasal Biopsies for Langerhans Cell Histiocytosis of the Hypothalamus: 2-Dimensional Operative Video. ⁶²	Bauman MMJ...Van Gompel JJ	36113147	2022
48	VE1 immunohistochemistry to determine BRAFV^600E mutation in Langerhans-cell histiocytosis. ⁶²	Casado-Lopez E...Perez-Martinez A	36192351	2022
49	Whole-body magnetic resonance imaging for staging Langerhans cell histiocytosis in children and young adults. ⁶²	Perrone A...Sieni E	36317710	2022
50	Multiple Lytic Bone Lesions Mimicking Langerhans Cell Histiocytosis: A Case of Infantile Mendelian Susceptibility to Mycobacterial Disease due to STAT1 Deficiency. ⁶²	He Y...Xiao W	36349275	2022

Sources

Genes for Histiocytosis

Genes related to Histiocytosis (0 elite genes):

(show all 26)

- Elite gene

- Cancer Census gene in COSMIC

#	Symbol	Description	Category	Score	Evidence	PubMed IDs
1	SLC29A3	Solute Carrier Family 29 Member 3	Protein Coding	33.47	DISEASES inferred ¹⁴ GeneCards inferred via : Disorders Publications Pathways & Interactions (show sections)
2	CD1A	CD1a Molecule	Protein Coding	27.21	DISEASES inferred ¹⁴ Novoseek inferred ⁵³ GeneCards inferred via : Publications (show sections)	17378241 12717600
3	CD207	CD207 Molecule	Protein Coding	26.39	DISEASES inferred ¹⁴ Novoseek inferred ⁵³ GeneCards inferred via : Publications (show sections)	17378241
4	SERPINA3	Serpin Family A Member 3	Protein Coding	18.64	DISEASES inferred ¹⁴ ¹⁴ GeneCards inferred via : Publications (show sections)
5	S100B	S100 Calcium Binding Protein B	Protein Coding	17.71	DISEASES inferred ¹⁴ Novoseek inferred ⁵³ GeneCards inferred via : Publications (show sections)	1282135
6	CD1C	CD1c Molecule	Protein Coding	16.89	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
7	CD101	CD101 Molecule	Protein Coding	15.64	GeneCards inferred via : Publications Expressions (show sections)
8	MAP2K1	Mitogen-Activated Protein Kinase Kinase 1	Protein Coding	15.3	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
9	BRAF	B-Raf Proto-Oncogene, Serine/Threonine Kinase	Protein Coding	15.26	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
10	CCR6	C-C Motif Chemokine Receptor 6	Protein Coding	15.15	DISEASES inferred ¹⁴ ¹⁴ GeneCards inferred via : Publications (show sections)
11	CD163	CD163 Molecule	Protein Coding	14.93	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
12	U2AF1	U2 Small Nuclear RNA Auxiliary Factor 1	Protein Coding	14.85	DISEASES inferred ¹⁴ ¹⁴ GeneCards inferred via : Publications (show sections)
13	TNFRSF8	TNF Receptor Superfamily Member 8	Protein Coding	14.12	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
14	ALK	ALK Receptor Tyrosine Kinase	Protein Coding	13.94	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
15	ARAF	A-Raf Proto-Oncogene, Serine/Threonine Kinase	Protein Coding	13.84	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
16	NRAS	NRAS Proto-Oncogene, GTPase	Protein Coding	13.64	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
17	STAB1	Stabilin 1	Protein Coding	13.49	DISEASES inferred ¹⁴ Novoseek inferred ⁵³	17229202
18	TNF	Tumor Necrosis Factor	Protein Coding	13.4	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
19	PTPRC	Protein Tyrosine Phosphatase Receptor Type C	Protein Coding	13.3	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
20	PECAM1	Platelet And Endothelial Cell Adhesion Molecule 1	Protein Coding	13.18	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
21	ITGAX	Integrin Subunit Alpha X	Protein Coding	13.16	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
22	IFNG	Interferon Gamma	Protein Coding	13.15	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
23	KIF5B	Kinesin Family Member 5B	Protein Coding	13.14	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
24	IL17A	Interleukin 17A	Protein Coding	12.95	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
25	CSF2	Colony Stimulating Factor 2	Protein Coding	12.89	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
26	IL4	Interleukin 4	Protein Coding	12.71	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)

Sources

Variations for Histiocytosis

Sources

Expression for Histiocytosis

Search GEO for disease gene expression data for Histiocytosis.

Sources

Pathways for Histiocytosis

Pathways directly related to Histiocytosis:

#	Pathway	Source
1	Defective SLC29A3 causes histiocytosis-lymphadenopathy plus syndrome (HLAS)	Reactome ⁶⁶

Pathways related to Histiocytosis according to GeneCards Suite gene sharing:

(show all 31)

#	Super pathways	Score	Top Affiliating Genes
1	Innate Immune System ⁶⁶ Show member pathways Immune System ⁶⁶ Neutrophil degranulation ⁶⁶	13.69	TNFRSF8 TNF SERPINA3 S100B PTPRC PECAM1
2	MIF Mediated Glucocorticoid Regulation ⁶⁴ Show member pathways all-trans-Retinoic Acid Signaling in Brain ⁶⁴ Endothelin-1 Signaling Pathway ⁶⁴ Glucocorticoid Receptor Signaling ⁶⁴ IL-6 Pathway ⁶⁴ MIF Regulation of Innate Immune Cells ⁶⁴ NFAT Signaling and Lymphocyte Interactions ⁶⁴ Overview of proinflammatory and profibrotic mediators ⁷⁴ PEDF Induced Signaling ⁶⁴ PGC1Alpha Pathway ⁶⁴ RAR-Gamma-RXR-Alpha Degradation ⁶⁴ STAT3 Pathway ⁶⁴	13.55	TNFRSF8 TNF NRAS MAP2K1 CCR6 BRAF
3	IL-9 Signaling Pathways ⁶⁵ Show member pathways Adipocytokines and Insulin Signaling ⁶⁵ Common Cytokine Receptor Gamma-Chain Family Signaling Pathways ⁶⁵ Development Thrombopoietin-regulated cell processes ²² IL-15 Signaling Pathways ⁶⁵ IL-2 Signaling Pathways ⁶⁵ IL-21 Signaling Pathways ⁶⁵ IL-4 Pathway ⁶⁴ IL-4 Signaling Pathways ⁶⁵ IL-7 Signaling Pathways ⁶⁵ IL-9 Pathway ⁶⁴ Immune response IL-9 signaling pathway ²² Interleukin-9 signaling ⁶⁶	12.77	TNF NRAS MAP2K1 BRAF ARAF
4	Toll-like receptor signaling pathway ⁷⁴ Show member pathways Hepatitis B infection ⁷⁴ Measles virus infection ⁷⁴ Regulation of toll-like receptor signaling pathway ⁷⁴	12.75	TNF NRAS MAP2K1 BRAF ARAF
5	Alzheimer's disease and miRNA effects ⁷⁴ Show member pathways Alzheimer's disease ⁷⁴	12.74	TNF NRAS MAP2K1 BRAF ARAF
6	Signaling by Receptor Tyrosine Kinases ⁶⁶	12.73	ALK BRAF MAP2K1 NRAS S100B
7	Endometrial cancer ⁷⁴ Show member pathways Chromosomal and microsatellite instability in colorectal cancer ⁷⁴ Melanoma ⁷⁴ Non-small cell lung cancer ⁷⁴ Pancreatic adenocarcinoma pathway ⁷⁴ Signal transduction PTEN pathway ²²	12.59	NRAS MAP2K1 BRAF ARAF ALK
8	4-1BB Pathway ⁶⁴ Show member pathways CD27 Pathway ⁶⁴ CD40 Signaling ⁶⁴ CMV and MAPK Pathways ⁶⁴ NF-KappaB Activation by EBV ⁶⁴ NF-kappaB Activation by Viruses ⁶⁴ PKR Pathway ⁶⁴ SUMO Pathway ⁶⁴ TNFR2 Pathway ⁶⁴	12.54	TNF NRAS MAP2K1 BRAF ARAF
9	Development EGFR signaling pathway ²² Show member pathways Development ERBB-family signaling ²² EGF Pathway ⁶⁴ EGFR Signaling Pathway ⁶⁷ ErbB receptor signaling network ⁶¹	12.39	NRAS MAP2K1 BRAF ARAF
10	Breast cancer pathway ⁷⁴ Show member pathways Embryonic stem cell pluripotency pathways ⁷⁴	12.37	NRAS MAP2K1 BRAF ARAF
11	G-protein signaling RAC1 in cellular process ²² Show member pathways CDC42 signaling events ⁶¹ Cytoskeleton remodeling CDC42 in cellular processes ²² RAC1 signaling pathway ⁶¹	12.28	NRAS MAP2K1 BRAF ARAF ALK
12	MAPK signaling pathway ⁷⁴	12.24	TNF NRAS MAP2K1 BRAF ARAF
13	Integrin-mediated cell adhesion ⁷⁴ Show member pathways HGF Pathway ⁶⁴	12.14	NRAS MAP2K1 BRAF ARAF
14	MAPK Signaling: Mitogens ⁶⁵ Show member pathways Akt Signaling Pathway ⁶⁵	12.13	NRAS MAP2K1 BRAF ARAF
15	NGF Pathway ⁶⁴ Show member pathways MAPK and NFkB signaling pathways inhibited by Yersinia YopJ ⁷⁴ TRKA Signaling ⁶⁴	12.06	ARAF BRAF MAP2K1 NRAS
16	Oncogenic MAPK signaling ⁶⁶ Show member pathways Signaling by BRAF and RAF1 fusions ⁶⁶	12.02	NRAS MAP2K1 BRAF ARAF
17	CCR3 Pathway in Eosinophils ⁶⁴ Show member pathways VEGF and S-1P Signaling ⁶⁴	11.99	NRAS MAP2K1 BRAF ARAF
18	Hematopoietic Stem Cells and Lineage-specific Markers ⁶⁵	11.86	TNF PTPRC CD1C CD163
19	MAP Kinase Signaling ³	11.83	NRAS MAP2K1 BRAF ARAF
20	TGF-beta Signaling Pathways ⁶⁵	11.81	MAP2K1 BRAF ARAF
21	MAPK Signaling: Oxidative Stress ⁶⁵	11.68	MAP2K1 BRAF ARAF
22	Innate Lymphoid Cells Differentiation ⁶⁵	11.64	TNF PTPRC CD101 CCR6
23	VEGF Pathway (Tocris) ⁷⁰ Show member pathways Sorafenib Pharmacodynamics ⁶⁰	11.6	ARAF BRAF MAP2K1 NRAS
24	Serotonin HTR1 group and FOS pathway ⁷⁴ Show member pathways Serotonin receptor 2 and ELK-SRF/GATA4 signaling ⁷⁴ Serotonin receptor 4/6/7 and NR3C signaling ⁷⁴	11.55	NRAS MAP2K1 BRAF
25	BDNF-TrkB signaling ⁷⁴ Show member pathways Synaptic signaling pathways associated with autism spectrum disorder ⁷⁴	11.54	NRAS MAP2K1 BRAF
26	Vemurafenib Pathway, Pharmacodynamics ⁶⁰	11.19	NRAS MAP2K1 BRAF ARAF
27	Aryl hydrocarbon receptor pathway ⁷⁴	11.16	TNF NRAS MAP2K1
28	Bladder cancer ⁷⁴	11.06	NRAS MAP2K1 BRAF ARAF
29	Dendritic Cells Developmental Lineage Pathway ⁶⁵	10.97	TNF PTPRC CD207 CD1C CD1A CD163
30	Crizotinib Pathway, Pharmacokinetics/Pharmacodynamics ⁶⁰	10.93	NRAS MAP2K1 BRAF ARAF ALK
31	MAPK pathway in congenital thyroid cancer ⁷⁴	10.59	MAP2K1 BRAF ALK

Sources

GO Terms for Histiocytosis

Cellular components related to Histiocytosis according to GeneCards Suite gene sharing:

#	Name	GO ID	Score	Top Affiliating Genes
1	membrane	GO:0016020	10.3	TNFRSF8 TNF STAB1 SLC29A3 PTPRC PECAM1
2	membrane	GO:0016021	10.3	TNFRSF8 TNF STAB1 SLC29A3 PTPRC PECAM1
3	external side of plasma membrane	GO:0009897	9.8	CCR6 CD163 CD1A CD1C CD207 PTPRC
4	plasma membrane	GO:0005886	9.75	TNF STAB1 PTPRC PECAM1 CD1C CD1A
5	plasma membrane	GO:0005887	9.75	TNF STAB1 PTPRC PECAM1 CD1C CD1A

Biological processes related to Histiocytosis according to GeneCards Suite gene sharing:

#	Name	GO ID	Score	Top Affiliating Genes
1	peptidyl-tyrosine phosphorylation	GO:0018108	9.88	PTPRC MAP2K1 BRAF ALK
2	antigen processing and presentation, exogenous lipid antigen via MHC class Ib	GO:0048007	9.73	CD1C CD1A
3	positive regulation of humoral immune response mediated by circulating immunoglobulin	GO:0002925	9.62	TNF PTPRC
4	antigen processing and presentation, endogenous lipid antigen via MHC class Ib	GO:0048006	9.56	CD1C CD1A
5	regulation of axon regeneration	GO:0048679	9.54	MAP2K1 BRAF
6	DN2 thymocyte differentiation	GO:1904155	9.46	CCR6 PTPRC
7	positive regulation of T cell mediated cytotoxicity	GO:0001916	9.43	PTPRC CD1C CD1A
8	MAPK cascade	GO:0000165	9.4	TNF PTPRC NRAS MAP2K1 BRAF ARAF