MCID: NNL001
MIFTS: 36

Non-Langerhans-Cell Histiocytosis malady

Categories: Rare diseases, Immune diseases

Aliases & Classifications for Non-Langerhans-Cell Histiocytosis

Aliases & Descriptions for Non-Langerhans-Cell Histiocytosis:

Name: Non-Langerhans-Cell Histiocytosis 12 50 14
Histiocytosis, Non-Langerhans-Cell 50 42 69

Classifications:



External Ids:

Disease Ontology 12 DOID:4330
MeSH 42 D015616
SNOMED-CT 64 127069007
UMLS 69 C0019624

Summaries for Non-Langerhans-Cell Histiocytosis

MalaCards based summary : Non-Langerhans-Cell Histiocytosis, also known as histiocytosis, non-langerhans-cell, is related to juvenile xanthogranuloma and reticulohistiocytic granuloma. An important gene associated with Non-Langerhans-Cell Histiocytosis is CD163 (CD163 Molecule), and among its related pathways/superpathways are Lysosome and LDL Oxidation in Atherogenesis. The drugs Busulfan and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include pituitary, and related phenotypes are cardiovascular system and cellular

Related Diseases for Non-Langerhans-Cell Histiocytosis

Diseases related to Non-Langerhans-Cell Histiocytosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 79)
id Related Disease Score Top Affiliating Genes
1 juvenile xanthogranuloma 32.5 ACTC1 CD163 DES F13A1
2 reticulohistiocytic granuloma 32.3 DES F13A1
3 erdheim-chester disease 11.6
4 histiocytosis, progressive mucinous 11.2
5 histiocytosis 10.6
6 dystrophic epidermolysis bullosa, nails only 10.3 ACTC1 F13A1
7 secondary adrenal insufficiency 10.3 ACTC1 F13A1
8 carbon monoxide-induced parkinsonism 10.3 ACTC1 DES
9 vagina leiomyosarcoma 10.3 ACTC1 DES
10 osteomyelitis 10.3 ACTC1 DES
11 myoma 10.3 ACTC1 DES
12 spleen angiosarcoma 10.3 ACTC1 DES
13 malignant dermis tumor 10.3 ACTC1 DES
14 urethral cancer 10.3 ACTC1 DES
15 biemond syndrome 10.3 ACTC1 DES
16 intestinal obstruction 10.2 ACTC1 DES
17 autosomal dominant tubulointerstitial kidney disease, ren-related 10.2 NPC1 SMPD1
18 pouchitis 10.2 NPC1 NPC2
19 5-fluorouracil poisoning 10.2 NPC1 NPC2
20 male urethral cancer 10.2 ACTC1 DES
21 neuroendocrine cell hyperplasia of infancy 10.2 NPC1 NPC2
22 pulmonary interstitial glycogenosis 10.2 NPC1 NPC2
23 atypical coarctation of aorta 10.2 NPC1 NPC2
24 pustulosis palmaris et plantaris 10.2 ACTC1 DES
25 cavernous sinus meningioma 10.2 CD163 DES F13A1
26 mediastinum liposarcoma 10.2 ACTC1 DES
27 coronary stenosis 10.2 ACTC1 DES
28 heart leiomyosarcoma 10.2 CD68 DES
29 congenital myasthenic syndrome 10.2 ACTC1 DES F13A1
30 glaucoma iridogoniodysgenesia 10.2 CD68 DES
31 dyslexia 10.2 ACTC1 DES F13A1
32 house allergic alveolitis 10.2 ACTC1 DES F13A1
33 diarrhea, chronic, with villous atrophy 10.2 ACTC1 DES F13A1
34 teeth hard tissue disease 10.2 CD163 CD68
35 sebaceous adenoma 10.2 ACTC1 DES
36 bilateral breast cancer 10.2 CD163 CD68
37 caroli disease 10.2 ACTC1 DES F13A1
38 maxillary sinus squamous cell carcinoma 10.2 CD163 CD68
39 glomangioma 10.2 ACTC1 DES
40 chiasmal syndrome 10.2 ACTC1 CD68
41 ulnar neuropathy 10.2 CD163 CD68
42 hemangioma of liver 10.2 ACTC1 CD68
43 omodysplasia 2 10.2 ACTC1 DES
44 bone dysplasia azouz type 10.1 ACTC1 SST
45 linear porokeratosis 10.1 ACTC1 CD68
46 oral erosive lichen 10.1 ACTC1 CD163 DES F13A1
47 neutrophil immunodeficiency syndrome 10.1 ACTC1 CD163 DES F13A1
48 47,xyy syndrome 10.1 NPC1 NPC2 SMPD1
49 episodic ataxia 10.1 ACTC1 CD68 DES
50 atrial septal defect 9 10.1 NPC1 NPC2 SMPD1

Graphical network of the top 20 diseases related to Non-Langerhans-Cell Histiocytosis:



Diseases related to Non-Langerhans-Cell Histiocytosis

Symptoms & Phenotypes for Non-Langerhans-Cell Histiocytosis

MGI Mouse Phenotypes related to Non-Langerhans-Cell Histiocytosis:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.97 ACTC1 CD163 DES F13A1 IL1RN NPC1
2 cellular MP:0005384 9.92 ACTC1 CD163 DES IL1RN NPC1 NPC2
3 hematopoietic system MP:0005397 9.91 SMPD1 CD163 CD207 CD68 F13A1 IL1RN
4 homeostasis/metabolism MP:0005376 9.9 ACTC1 CD163 DES F13A1 HEXA IL1RN
5 immune system MP:0005387 9.61 CD163 CD207 CD68 IL1RN NPC1 NPC2
6 mortality/aging MP:0010768 9.28 F13A1 HEXA IL1RN NPC1 NPC2 SMPD1

Drugs & Therapeutics for Non-Langerhans-Cell Histiocytosis

Drugs for Non-Langerhans-Cell Histiocytosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 124)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Busulfan Approved, Investigational Phase 3,Phase 2 55-98-1 2478
2
Cyclophosphamide Approved, Investigational Phase 3,Phase 2 50-18-0, 6055-19-2 2907
3
Cyclosporine Approved, Investigational, Vet_approved Phase 3,Phase 2,Phase 1 79217-60-0, 59865-13-3 5284373 6435893
4
Dexamethasone Approved, Investigational, Vet_approved Phase 3,Phase 2 50-02-2 5743
5
Etoposide Approved Phase 3,Phase 2 33419-42-0 36462
6
Hydrocortisone Approved, Vet_approved Phase 3,Phase 2 50-23-7 5754 657311
7
Methotrexate Approved Phase 3,Phase 2 1959-05-2, 59-05-2 126941
8
Miconazole Approved, Investigational, Vet_approved Phase 3,Phase 2,Phase 1 22916-47-8 4189
9
Mycophenolate mofetil Approved, Investigational Phase 3,Phase 2,Phase 1 128794-94-5 5281078
10
Mycophenolic acid Approved Phase 3,Phase 2,Phase 1 24280-93-1 446541
11
Doxorubicin Approved, Investigational Phase 3,Phase 2 23214-92-8 31703
12
Miglustat Approved Phase 3,Phase 2,Phase 1 72599-27-0 51634
13
Methylprednisolone Approved, Vet_approved Phase 2, Phase 3, Phase 1 83-43-2 6741
14
Prednisolone Approved, Vet_approved Phase 2, Phase 3, Phase 1 50-24-8 5755
15
Asparaginase Approved Phase 3 9015-68-3
16
Pegaspargase Approved, Investigational Phase 3 130167-69-0
17
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3,Phase 2 59-30-3 6037
18
leucovorin Approved, Nutraceutical Phase 3,Phase 2 58-05-9 54575, 6560146 143
19
Butyric Acid Experimental Phase 3,Phase 2 107-92-6 264
20
Doxil Approved June 1999 Phase 3,Phase 2 31703
21
1-Deoxynojirimycin Experimental Phase 3,Phase 2 19130-96-2 1374
22 Alkylating Agents Phase 3,Phase 2
23 Anti-Bacterial Agents Phase 3,Phase 2
24 Antibiotics, Antitubercular Phase 3,Phase 2
25 Antiemetics Phase 3,Phase 2,Phase 1
26 Antifungal Agents Phase 3,Phase 2,Phase 1
27 Anti-Infective Agents Phase 3,Phase 2,Phase 1
28 Anti-Inflammatory Agents Phase 3,Phase 2,Phase 1
29 Antilymphocyte Serum Phase 3,Phase 2
30 Antimetabolites Phase 3,Phase 2
31 Antimetabolites, Antineoplastic Phase 3,Phase 2
32 Antineoplastic Agents, Alkylating Phase 3,Phase 2
33 Antineoplastic Agents, Hormonal Phase 3,Phase 2,Phase 1
34 Antineoplastic Agents, Phytogenic Phase 3,Phase 2
35 Antirheumatic Agents Phase 3,Phase 2,Phase 1
36 Autonomic Agents Phase 3,Phase 2,Phase 1
37 BB 1101 Phase 3,Phase 2
38 Calcineurin Inhibitors Phase 3,Phase 2,Phase 1
39 Cortisol succinate Phase 3,Phase 2
40 Dermatologic Agents Phase 3,Phase 2,Phase 1
41 Dexamethasone 21-phosphate Phase 3,Phase 2
42 Dexamethasone acetate Phase 3,Phase 2 1177-87-3
43 Etoposide phosphate Phase 3,Phase 2
44 Folic Acid Antagonists Phase 3,Phase 2
45 Gastrointestinal Agents Phase 3,Phase 2,Phase 1
46 glucocorticoids Phase 3,Phase 2,Phase 1
47 Hormone Antagonists Phase 3,Phase 2,Phase 1
48 Hormones Phase 3,Phase 2,Phase 1
49 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3,Phase 2,Phase 1
50 Hydrocortisone 17-butyrate 21-propionate Phase 3,Phase 2

Interventional clinical trials:

(show top 50) (show all 71)
id Name Status NCT ID Phase
1 Combination Chemotherapy Followed By Donor Stem Cell Transplant in Treating Patients With Hemophagocytic Lymphohistiocytosis Unknown status NCT00334672 Phase 3
2 T-Cell Depletion and Stem Cell Transplant for Immune Deficiencies and Histiocytic Disorders Unknown status NCT00176826 Phase 2, Phase 3
3 Application of Miglustat in Patients With Niemann-Pick Type C Completed NCT01760564 Phase 3
4 Stem Cell Transplant for Inborn Errors of Metabolism Completed NCT00176904 Phase 2, Phase 3
5 L-DEP as an Initial Treatment for EBV-HLH Recruiting NCT02912702 Phase 3
6 Splenectomy as a Treatment for Patient With Relapsed Haemophagocytic Lymphohistiocytosis of Unknown Etiology Recruiting NCT02862054 Phase 2, Phase 3
7 L-DEP Regimen as a Salvage Therapy for Refractory Epstein Barr Virus-induced Hemophagocytic Lymphohistiocytosis Recruiting NCT02631109 Phase 3
8 A Study to Investigate the Safety and Efficacy of an Anti-IFNγ mAb in Children Affected by Primary Haemophagocytic Lymphohistiocytosis Recruiting NCT01818492 Phase 2, Phase 3
9 Study of VTS-270 (2-hydroxypropyl-β-cyclodextrin) to Treat Niemann-Pick Type C1 (NPC1) Disease Recruiting NCT02534844 Phase 2, Phase 3
10 Efficacy, Safety, Pharmacodynamic, and Pharmacokinetics Study of Olipudase Alfa in Patients With Acid Sphingomyelinase Deficiency Recruiting NCT02004691 Phase 2, Phase 3
11 Treatment Protocol for Hemophagocytic Lymphohistiocytosis 2004 Active, not recruiting NCT00426101 Phase 3
12 Arimoclomol Prospective Study in Patients Diagnosed With NiemannPick Disease Type C Active, not recruiting NCT02612129 Phase 2, Phase 3
13 Trial of DA-EPOCH Regimen for NHL With HLH Unknown status NCT01818908 Phase 2
14 Hybrid Immunotherapy for Hemophagocytic LymphoHistiocytosis Completed NCT01104025 Phase 2
15 Stem Cell Transplant for Immunologic or Histiocytic Disorders Completed NCT00176865 Phase 2
16 Phase 1/2 Study of Vorinostat Therapy in Niemann-Pick Disease, Type C1 Completed NCT02124083 Phase 1, Phase 2
17 Biomarker Validation for Niemann-Pick Disease, Type C: Safety and Efficacy of N-Acetyl Cysteine Completed NCT00975689 Phase 1, Phase 2
18 Unrelated Hematopoietic Stem Cell Transplantation(HSCT) for Genetic Diseases of Blood Cells Completed NCT00730314 Phase 1, Phase 2
19 Miglustat in Niemann-Pick Type C Disease Completed NCT00517153 Phase 2
20 Treatment of Familiar Lymphohistiocytosis Recruiting NCT02472054 Phase 1, Phase 2
21 Alemtuzumab in Combination With Etoposide and Dexamethasone for the Treatment of Adult Patients With Hemophagocytic Lymphohistiocytosis Recruiting NCT02385110 Phase 2
22 Tocilizumab and Hemophagocytic Lymphohistiocytosis (HLH) Recruiting NCT02007239 Phase 2
23 A Study of Lenalidomide for Adult Histiocyte Disorders Recruiting NCT02523040 Phase 2
24 Study of Pharmacokinetics and Preliminary Efficacy in Patients With Niemann-Pick C1 Recruiting NCT02912793 Phase 1, Phase 2
25 Safety, Tolerability, PK, and Efficacy Evaluation of Repeat Ascending Doses of Olipudase Alfa in Pediatric Patients <18 Years of Age With Acid Sphingomyelinase Deficiency Recruiting NCT02292654 Phase 1, Phase 2
26 Immune Disorder HSCT Protocol Recruiting NCT01821781 Phase 2
27 T Cell Depletion for Recipients of HLA Haploidentical Related Donor Stem Cell Grafts Recruiting NCT00368355 Phase 2
28 Reduced Intensity Conditioning for Hemophagocytic Syndromes or Selected Primary Immune Deficiencies (BMT CTN 1204) Active, not recruiting NCT01998633 Phase 2
29 Phase I/II Pilot Study of Mixed Chimerism to Treat Inherited Metabolic Disorders Active, not recruiting NCT01372228 Phase 1, Phase 2
30 A Long-Term Study of Olipudase Alfa in Patients With Acid Sphingomyelinase Deficiency Enrolling by invitation NCT02004704 Phase 2
31 Pediatric MATCH: Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients With Advanced Refractory Solid Tumors or Lymphomas Not yet recruiting NCT03155620 Phase 2
32 Dabrafenib and Trametinib in People With BRAF V600E Mutation Positive Lesions in Erdheim Chester Disease Suspended NCT02281760 Phase 2
33 Pilot Study of Tocilizumab in Patients With Erdheim-Chester Disease Terminated NCT01727206 Phase 2
34 Hematopoietic Stem Cell Transplantation (HCT) for Inborn Errors of Metabolism Terminated NCT00668564 Phase 2
35 Hydroxypropyl Beta Cyclodextrin for Niemann-Pick Type C1 Disease Completed NCT01747135 Phase 1
36 Tolerability and Safety Study of Recombinant Human Acid Sphingomyelinase in Acid Sphingomyelinase Deficiency Patients Completed NCT01722526 Phase 1
37 Saccadic Eye Movements in Patients With Niemann-Pick Type C Disease Completed NCT00316498 Phase 1
38 Safety Study of Gene Modified Donor T Cell Infusion After Stem Cell Transplant for Non-Malignant Diseases Recruiting NCT02231710 Phase 1
39 Study of the Pharmacokinetics of Trappsol and Effects on Potential Biomarkers of Niemann-Pick C1 (NPC1) Recruiting NCT02939547 Phase 1
40 UCB Transplant of Inherited Metabolic Diseases With Administration of Intrathecal UCB Derived Oligodendrocyte-Like Cells Recruiting NCT02254863 Phase 1
41 BMT Abatacept for Non-Malignant Diseases Recruiting NCT01917708 Phase 1
42 Human Placental-Derived Stem Cell Transplantation Recruiting NCT01586455 Phase 1
43 Administration of Donor T Cells With the Caspase-9 Suicide Gene Active, not recruiting NCT01494103 Phase 1
44 Safety Study of rhASM Enzyme Replacement Therapy in Adults With Acid Sphingomyelinase Deficiency (Niemann-Pick Disease) Terminated NCT00410566 Phase 1
45 New Candidate Criteria for Diagnosis of Macrophage Activation Syndrome Unknown status NCT01095146
46 Pilot Study of Unrelated Donor Hematopoietic Stem Cell Transplantation in Patients With Life Threatening Hemophagocytic Disorders Unknown status NCT00006056
47 Study of Pulmonary Complications in Pediatric Patients With Storage Disorders Undergoing Allogeneic Hematopoietic Stem Cell Transplantation Unknown status NCT00005900
48 Secondary Adult's Hemophagocytic Lymphohistiocytosis and Innate Immunity Completed NCT01125319
49 The Prognostic and Diagnostic Value of Ferritin in Critically Ill Patients With Special Focus on Underlying Hemophagocytic Lymphohistiocytosis Completed NCT02854943
50 A Prospective Non-therapeutic Study in Patients Diagnosed With Niemann-Pick Disease Type C Completed NCT02435030

Search NIH Clinical Center for Non-Langerhans-Cell Histiocytosis

Cochrane evidence based reviews: histiocytosis, non-langerhans-cell

Genetic Tests for Non-Langerhans-Cell Histiocytosis

Anatomical Context for Non-Langerhans-Cell Histiocytosis

MalaCards organs/tissues related to Non-Langerhans-Cell Histiocytosis:

39
Pituitary

Publications for Non-Langerhans-Cell Histiocytosis

Articles related to Non-Langerhans-Cell Histiocytosis:

(show all 24)
id Title Authors Year
1
Non-Langerhans cell histiocytosis. ( 26739442 )
2016
2
Spontaneous regression of infantile dural-based non-Langerhans cell histiocytosis after surgery: case report. ( 25634822 )
2015
3
Suprasellar non-Langerhans cell histiocytosis (Erdheim-Chester disease)--a case report. ( 23465991 )
2013
4
Retinal manifestations in a patient with xanthoma disseminatum (non-langerhans cell histiocytosis). ( 21484660 )
2011
5
Rare case of infratentorial non-Langerhans cell histiocytosis with lethal outcome presenting multiple diagnostic difficulties. ( 22025140 )
2011
6
Non-Langerhans cell histiocytosis with isolated CNS involvement: an unusual variant of Erdheim-Chester disease. ( 20337948 )
2010
7
Endolymphatic non-langerhans cell histiocytosis of the larynx: report of an uncommon disease manifestation. ( 20463609 )
2010
8
Intracranial non-Langerhans cell histiocytosis presenting as an isolated intraparenchymal lesion. ( 20635081 )
2010
9
Fibroblastic rheumatism: fibromatosis rather than non-Langerhans cell histiocytosis. ( 19615014 )
2010
10
Diffuse xanthogranulomatous dermatitis and systemic Langerhans cell histiocytosis: A novel case that demonstrates bridging between non-Langerhans cell histiocytosis and Langerhans cell histiocytosis. ( 19022530 )
2009
11
Non-Langerhans cell histiocytosis in a child with Kawasaki disease. ( 21686403 )
2009
12
Cerebral non-Langerhans cell histiocytosis in a 17-year-old patient with a headache: case report. ( 19363626 )
2009
13
Successful treatment of Erdheim-Chester disease, a non-Langerhans-cell histiocytosis, with interferon-alpha. ( 16020507 )
2005
14
[Erdheim-Chester disease: a non-Langerhans cell histiocytosis. A clinical-case and review of the literature]. ( 15344569 )
2004
15
Primary cerebral non-Langerhans cell histiocytosis: MRI and differential diagnosis. ( 12221448 )
2002
16
Problems in differential diagnosis of non Langerhans cell histiocytosis with pituitary involvement: case report and review of literature. ( 12665654 )
2002
17
A child with both Langerhans and non-Langerhans cell histiocytosis. ( 12383099 )
2002
18
Progressive histiocytosis: description of a case of slow-course non-Langerhans cell histiocytosis. ( 12399681 )
2002
19
Benign cephalic histiocytosis progressing into juvenile xanthogranuloma: a non-Langerhans cell histiocytosis transforming under the influence of a virus? ( 10698221 )
2000
20
Non-Langerhans cell histiocytosis associated with lymphocyte-predominant Hodgkin's disease. ( 10564322 )
1999
21
Generalized non-Langerhans cell histiocytosis: four cases illustrate a spectrum of disease. ( 7737766 )
1995
22
A unique non-Langerhans cell histiocytosis with some features of generalized eruptive histiocytoma. ( 8034797 )
1994
23
An unusual case of non-Langerhans cell histiocytosis. ( 8294600 )
1994
24
Congenital self-healing non-Langerhans cell histiocytosis. ( 3058480 )
1988

Variations for Non-Langerhans-Cell Histiocytosis

Expression for Non-Langerhans-Cell Histiocytosis

Search GEO for disease gene expression data for Non-Langerhans-Cell Histiocytosis.

Pathways for Non-Langerhans-Cell Histiocytosis

Pathways related to Non-Langerhans-Cell Histiocytosis according to GeneCards Suite gene sharing:

id Super pathways Score Top Affiliating Genes
1 11.11 CD68 HEXA NPC1 NPC2 SMPD1
2 10.36 CD68 SMPD1
3
Show member pathways
10.1 NPC1 NPC2

GO Terms for Non-Langerhans-Cell Histiocytosis

Cellular components related to Non-Langerhans-Cell Histiocytosis according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.7 CD163 F13A1 IL1RN NPC1 NPC2 SMPD1
2 lysosome GO:0005764 9.26 HEXA NPC1 NPC2 SMPD1
3 lysosomal lumen GO:0043202 8.8 HEXA NPC2 SMPD1

Biological processes related to Non-Langerhans-Cell Histiocytosis according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 response to cocaine GO:0042220 9.4 PITX3 SMPD1
2 muscle filament sliding GO:0030049 9.37 ACTC1 DES
3 low-density lipoprotein particle clearance GO:0034383 9.32 NPC1 NPC2
4 cholesterol efflux GO:0033344 9.26 NPC1 NPC2
5 response to drug GO:0042493 9.26 ACTC1 NPC1 SMPD1 SST
6 cholesterol transport GO:0030301 9.16 NPC1 NPC2
7 receptor-mediated endocytosis GO:0006898 8.92 CD163 CD207 NPC1 NPC2

Sources for Non-Langerhans-Cell Histiocytosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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