MCID: NNL001
MIFTS: 35

Non-Langerhans-Cell Histiocytosis

Categories: Bone diseases, Immune diseases, Rare diseases, Skin diseases

Aliases & Classifications for Non-Langerhans-Cell Histiocytosis

MalaCards integrated aliases for Non-Langerhans-Cell Histiocytosis:

Name: Non-Langerhans-Cell Histiocytosis 12 20 15
Histiocytosis, Non-Langerhans-Cell 73 20 44 70
Non-Langerhans Cell Histiocytosis 58

Classifications:

Orphanet: 58  
Rare systemic and rhumatological diseases
Rare skin diseases


External Ids:

Disease Ontology 12 DOID:4330
MeSH 44 D015616
SNOMED-CT 67 127069007
MESH via Orphanet 45 D015616
ICD10 via Orphanet 33 D76.3
UMLS via Orphanet 71 C0019624
Orphanet 58 ORPHA157987
UMLS 70 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 erdheim-chester disease. An important gene associated with Non-Langerhans-Cell Histiocytosis is CD163 (CD163 Molecule), and among its related pathways/superpathways are Tight junction and Immunoregulatory interactions between a Lymphoid and a non-Lymphoid cell. Affiliated tissues include bone, skin and myeloid.

Wikipedia : 73 Non-Langerhans cell histiocytosis refers to a family of histiocytosis characterized by the absence of... more...

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 196)
# Related Disease Score Top Affiliating Genes
1 juvenile xanthogranuloma 31.9 SERPINA3 F13A1 CD1E CD1A
2 erdheim-chester disease 31.1 SERPINA3 CD1E CD1D CD1C CD1B CD1A
3 progressive nodular histiocytosis 30.8 F13A1 ACTC1
4 fibromatosis 30.6 DES ACTC1
5 reticulohistiocytic granuloma 30.3 SERPINA3 CD68 CD1E CD1D CD1C CD1B
6 fibroblastic rheumatism 30.2 F13A1 DES CD163 ACTC1
7 histiocytosis 30.1 U2AF1 SERPINA3 CD207 CD1C CD1A CD163
8 pigmented villonodular synovitis 29.9 SERPINA3 CD68
9 langerhans cell histiocytosis 29.6 U2AF1 F13A1 CD68 CD207 CD1C CD1A
10 skin disease 29.2 U2AF1 SERPINA3 F13A1 CD1C CD1A CD163
11 exophthalmos 28.8 CD68 CD1E CD1D CD1C CD1B CD1A
12 histiocytosis, progressive mucinous 11.3
13 diabetes insipidus 10.4
14 ovarian fibrothecoma 10.3 DES ACTC1
15 rosai-dorfman disease 10.3
16 scleromyxedema 10.3 F13A1 ACTC1
17 adult cystic nephroma 10.3 DES ACTC1
18 benign metastasizing leiomyoma 10.3 DES ACTC1
19 pulmonary vein stenosis 10.3 DES ACTC1
20 botryoid rhabdomyosarcoma 10.3 DES ACTC1
21 intravascular papillary endothelial hyperplasia 10.3 SERPINA3 ACTC1
22 parachordoma 10.3 DES ACTC1
23 scrotum neoplasm 10.3 SERPINA3 ACTC1
24 glomangioma 10.3 DES ACTC1
25 spindle cell lipoma 10.3 DES ACTC1
26 malignant fibrous histiocytoma of bone 10.3 SERPINA3 DES
27 reticulum cell sarcoma 10.3 DES ACTC1
28 sarcoidosis 1 10.3
29 xanthoma disseminatum 10.3
30 ossifying fibromyxoid tumor 10.3 DES ACTC1
31 type c thymoma 10.3 SST SERPINA3
32 binswanger's disease 10.3 SST ACTC1
33 erythema elevatum diutinum 10.3 F13A1 CD1E
34 malignant mesenchymoma 10.3 SERPINA3 DES
35 chondromyxoid fibroma 10.3 DES ACTC1
36 skull base cancer 10.3 SST SERPINA3
37 embryonal sarcoma 10.3 SERPINA3 DES
38 skull base meningioma 10.2 SST SERPINA3
39 predominantly cortical thymoma 10.2 CD1E CD1A
40 tolosa-hunt syndrome 10.2 CD1E CD1A
41 dendritic cell thymoma 10.2 CD1E CD1A
42 multifocal motor neuropathy 10.2 CD1E CD1A
43 angiolipoma 10.2 F13A1 DES ACTC1
44 rare tumor 10.2 SST DES ACTC1
45 exanthem 10.2
46 benign cephalic histiocytosis 10.2
47 oral hairy leukoplakia 10.2 CD1E CD1A
48 leiomyosarcoma 10.2 SERPINA3 DES ACTC1
49 syringocystadenoma papilliferum 10.2 SERPINA3 DES ACTC1
50 epithelioid leiomyosarcoma 10.2 SERPINA3 DES ACTC1

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

Drugs & Therapeutics for Non-Langerhans-Cell Histiocytosis

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 International Rare Histiocytic Disorders Registry (IRHDR) Recruiting NCT02285582

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:

40
Bone, Skin, Myeloid, Thymus, Bone Marrow, Spinal Cord, Pituitary

Publications for Non-Langerhans-Cell Histiocytosis

Articles related to Non-Langerhans-Cell Histiocytosis:

(show top 50) (show all 376)
# Title Authors PMID Year
1
Intracranial juvenile xanthogranuloma in an infant. 61
33660104 2021
2
Dermoscopic follow-up of cutaneous rosai-dorfman: spontaneous regression. 61
33771755 2021
3
Isolated thoracic intramedullary Erdheim-Chester disease presenting with paraplegia: a case report and literature review. 61
33711983 2021
4
Autopsy case of Rosai-Dorfman disease presenting as fibrinous pericarditis. 61
33227653 2021
5
Central Nervous System Complications of Erdheim-Chester Disease: FDG PET/CT Findings. 61
33577201 2021
6
Pregnant patient with extranodal CNS Rosai-Dorfman disease with new-onset seizures: the conundrum of differentiating disease progression from eclampsia. 61
33602767 2021
7
Non-Langerhans cell histiocytosis xanthogranuloma patient with varying histopathology leading to progressive nodular histiocytosis. 61
33586878 2021
8
X-linked chronic granulomatous disease misdiagnosed as non-Langerhans cell histiocytosis: A case report. 61
32697334 2021
9
Case of Generalized Tumor-Type Rosai-Dorfman Disease With Sarcoidosis-Like Histological Features and IgG4-Positive Plasma Cells. 61
33337631 2021
10
Indeterminate Cell Histiocytosis Mimicking Rosacea. 61
33628700 2021
11
Oral Findings of Erdheim-Chester Disease: A Case Report and Review of the Literature. 61
33582038 2021
12
Erdheim-Chester Disease: Two cases from an ophthalmic perspective. 61
33204897 2020
13
Erdheim-Chester disease: a rapidly evolving disease model. 61
32591646 2020
14
Successful Salvage Treosulfan-Based Megachemotherapy With Allogeneic Stem Cell Transplantation in Nonsyndromic, Therapy-Resistant Disseminated Juvenile Xanthogranuloma: A Case Report. 61
32684371 2020
15
Solitary long-bone epiphyseal lesions in children: radiologic-pathological correlation and epidemiology. 61
32638051 2020
16
Synovial Non-langerhans Cell Histiocytosis of the Shoulder: A Case Report and Review of the Literature. 61
33708700 2020
17
Disseminated non-Langerhans cell histiocytosis with an IRF2BP2-NTRK1 gene fusion identified by next-generation sequencing. 61
33134460 2020
18
MAP2K1-driven mixed Langerhans cell histiocytosis, Rosai-Dorfman-Destombes disease and Erdheim-Chester disease, clonally related to acute myeloid leukemia. 61
33188581 2020
19
Prevalence of Hypothyroidism in Patients With Erdheim-Chester Disease. 61
33119105 2020
20
Isolated intraocular histiocytosis-A rarely reported entity masquerading clinically as uveitis. 61
32823479 2020
21
Clinical presentation, imaging and response to interferon-alpha therapy in Erdheim-Chester disease: case-based review. 61
32572610 2020
22
Solitary juvenile xanthogranuloma of the hypopharynx. Clinico-pathologic study in a child with β-Thalassemia Major and Cutaneous Mastocytosis. 61
32505931 2020
23
Multiple Xanthogranulomas in a Teenager. 61
32884872 2020
24
Juvenile xanthogranulomas: Examining single, multiple, and extracutaneous presentations. 61
32468628 2020
25
Juvenile xanthogranuloma of the subdural spaces mimicking chronic hematoma with positive FDG uptake on PET: case report. 61
32679560 2020
26
Retroperitoneal Erdheim-Chester disease without skeletal bone involvement mimicking uterine sarcoma with multiple organ involvement. 61
32689779 2020
27
Solitary adult xanthogranuloma in external auditory canal: Cyto-histopathological correlation of an uncommon entity at an uncommon site. 61
32275346 2020
28
Long-term outcomes of children with extracutaneous juvenile xanthogranulomas in Japan. 61
32383820 2020
29
Rosai-Dorfman Disease-Utility of 18F-FDG PET/CT for Initial Evaluation and Follow-up. 61
32349088 2020
30
Therapy Response in a Pediatric Patient With Extracutaneous Juvenile Xanthogranuloma Monitored by FDG PET/CT. 61
32049719 2020
31
Erdheim-Chester Disease With Extensive Pericardial Involvement: A Case Report and Systematic Review. 61
32256919 2020
32
A Multicenter Cross-Sectional Study and Systematic Review of Necrobiotic Xanthogranuloma With Proposed Diagnostic Criteria. 61
31940000 2020
33
Uncommon cutaneous non-Langerhans cell histiocytosis arising from dermal dendritic cells in adults: a clinicopathological study of five cases. 61
32250254 2020
34
Erdheim-Chester disease: expanding the spectrum of cutaneous manifestations. 61
31120137 2020
35
Real-World Toxicity Experience with BRAF/MEK Inhibitors in Patients with Erdheim-Chester Disease. 61
32043767 2020
36
Erdheim-Chester Disease: A Case Report and Review of the Literature. 61
32637228 2020
37
Erdheim-Chester Disease: A Rare Clinical Entity. 61
32908822 2020
38
The Rare Case of an Adult-Onset Xanthogranuloma of the Paranasal Sinuses: A Histological Dilemma. 61
32566345 2020
39
Unique Presentation of Rosai-Dorfman Disease as Concomitant Appendiceal and Rectal Masses with IgG4-Positive Plasma Cells Diagnosed by Core Needle Biopsy. 61
33133711 2020
40
Disseminated Juvenile Xanthogranuloma and Hemophagocytic Lymphohistiocytosis Developed During Treatment of Acute Lymphoblastic Leukemia: Case Report. 61
32719740 2020
41
Juvenile Xanthogranuloma: Case Report and Literature Review. 61
32158278 2020
42
Clinicopathological features, treatment approaches, and outcomes in Rosai-Dorfman disease. 61
31004029 2020
43
Cardiothoracic manifestations of Erdheim-Chester disease. 61
31386554 2019
44
Head and Neck Sinus Histiocytosis with Massive Lymphadenopathy Radiology-Pathology Correlation. 61
29855802 2019
45
Erdheim-Chester disease progression from miliary pulmonary nodules to large tumours. 61
31463063 2019
46
Erdheim-Chester disease mimicking lumbar nerve schwannoma: case report and literature review. 61
33122653 2019
47
Skull Base Manifestations of Erdheim-Chester Disease: A Case Series and Systematic Review. 61
30828728 2019
48
[Histiocytosis in the dermatological context of the new classification]. 61
31414152 2019
49
Fine-needle aspiration specimens of 3 cases of intra-abdominal Rosai-Dorfman disease with comprehensive review of the literature. 61
31272602 2019
50
Scleritis and anterior uveitis may herald the development of an epibulbar tumor in patients with extranodal Rosai-Dorfman disease: a case report. 61
31291929 2019

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

GO Terms for Non-Langerhans-Cell Histiocytosis

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

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.86 SST SERPINA3 CD1E CD1D CD1C CD1B
2 lysosome GO:0005764 9.72 CD68 CD1E CD1D CD1C CD1B
3 endosome GO:0005768 9.63 CD68 CD1E CD1D CD1C CD1B CD1A
4 blood microparticle GO:0072562 9.54 SERPINA3 F13A1 ACTC1
5 endosome membrane GO:0010008 9.35 CD68 CD1D CD1C CD1B CD1A
6 external side of plasma membrane GO:0009897 9.1 CD1E CD1D CD1C CD1B CD1A CD163

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

# Name GO ID Score Top Affiliating Genes
1 immune system process GO:0002376 9.77 CD1E CD1D CD1C CD1B CD1A
2 immune response GO:0006955 9.72 CD1E CD1D CD1C CD1B CD1A
3 adaptive immune response GO:0002250 9.71 CD1E CD1C CD1B CD1A
4 regulation of immune response GO:0050776 9.56 CD1D CD1C CD1B CD1A
5 positive regulation of T cell mediated cytotoxicity GO:0001916 9.55 CD1E CD1D CD1C CD1B CD1A
6 antigen processing and presentation, exogenous lipid antigen via MHC class Ib GO:0048007 9.35 CD1E CD1D CD1C CD1B CD1A
7 antigen processing and presentation, endogenous lipid antigen via MHC class Ib GO:0048006 9.02 CD1E CD1D CD1C CD1B CD1A

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

# Name GO ID Score Top Affiliating Genes
1 lipopeptide binding GO:0071723 9.55 CD1E CD1D CD1C CD1B CD1A
2 exogenous lipid antigen binding GO:0030884 9.35 CD1E CD1D CD1C CD1B CD1A
3 endogenous lipid antigen binding GO:0030883 9.02 CD1E CD1D CD1C CD1B CD1A

Sources for Non-Langerhans-Cell Histiocytosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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