MCID: HST010
MIFTS: 49

Histiocytosis

Categories: Immune diseases
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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:



External Ids:

Disease Ontology 11 DOID:3405
MeSH 43 D015614
NCIt 49 C3106
SNOMED-CT 68 60657004
UMLS 71 C0019618 C0019621 C0398597

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...

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:



Diseases related to Histiocytosis

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

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
1
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
2
Mycophenolic acid Approved, Investigational Phase 4 24280-93-1 446541
3 Antineoplastic Agents, Hormonal Phase 4
4 Anti-Bacterial Agents Phase 4
5 Anti-Infective Agents Phase 4
6 Hormones Phase 4
7 Hormone Antagonists Phase 4
8 glucocorticoids Phase 4
9 Antitubercular Agents Phase 4
10 Anti-Inflammatory Agents Phase 4
11 Antibiotics, Antitubercular Phase 4
12
Vinblastine Approved Phase 3 865-21-4 241903 13342
13
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
14
Prednisolone acetate Approved, Vet_approved Phase 3 52-21-1
15
Prednisolone Approved, Vet_approved Phase 3 50-24-8 4894 5755
16
Mercaptopurine Approved Phase 3 50-44-2 667490
17
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5 1875
18
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 4159 6741
19
Cytarabine Approved, Investigational Phase 3 147-94-4 6253
20
Levoleucovorin Approved, Experimental, Investigational Phase 2, Phase 3 68538-85-2, 58-05-9, 73951-54-9 149436 6006
21
Methotrexate Approved Phase 2, Phase 3 1959-05-2, 59-05-2 4112 126941
22
Vincristine Approved, Investigational Phase 3 2068-78-2, 57-22-7 5978
23
Cladribine Approved, Investigational Phase 2, Phase 3 4291-63-8 20279
24
Indomethacin Approved, Investigational Phase 2, Phase 3 53-86-1 3715
25
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
26
Busulfan Approved, Investigational Phase 2, Phase 3 55-98-1 2478
27
Folic acid Approved, Nutraceutical, Vet_approved Phase 2, Phase 3 59-30-3 6037
28
Prednisolone hemisuccinate Experimental Phase 3 2920-86-7 4897
29 Vitamins Phase 3
30 Trace Elements Phase 3
31 Antidotes Phase 3
32 Micronutrients Phase 3
33 Liver Extracts Phase 3
34 Antiviral Agents Phase 3
35 Antimitotic Agents Phase 3
36 Tubulin Modulators Phase 3
37
Methylprednisolone Acetate Phase 3 584547
38 Antirheumatic Agents Phase 2, Phase 3
39 Folic Acid Antagonists Phase 2, Phase 3
40 Folate Phase 2, Phase 3
41 Vitamin B9 Phase 2, Phase 3
42 Vitamin B Complex Phase 2, Phase 3
43 Dermatologic Agents Phase 2, Phase 3
44 Immunoglobulins, Intravenous Phase 2, Phase 3
45 Immunoglobulins Phase 2, Phase 3
46 Antibodies Phase 2, Phase 3
47 gamma-Globulins Phase 2, Phase 3
48 Rho(D) Immune Globulin Phase 2, Phase 3
49 Anti-Inflammatory Agents, Non-Steroidal Phase 2, Phase 3
50 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

Genetic Tests for Histiocytosis

Anatomical Context for Histiocytosis

Organs/tissues related to Histiocytosis:

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

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
12717600 2003
2
Oral submucosal dendrocytes: factor XIIIa+ and CD34+ dendritic cell populations in normal tissue and fibrovascular lesions. 53 62
1282135 1992
3
A case of renal involvement in juvenile xanthogranulomatosis. 62
36439921 2023
4
Rosai-Dorfman disease presenting as stridor and hoarseness in a young female patient. 62
36452890 2023
5
Outcomes of liver transplantation in children with Langerhans cell histiocytosis: Experience from a quaternary care center. 62
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
36266951 2023
7
PET/CT Showing a Case of Langerhans Cell Histiocytosis Involving the Pleura. 62
36469071 2023
8
Histiocytic Diseases. 62
36270835 2023
9
Langerhans Cell Histiocytosis Appearing as Wandering Pulmonary Nodules. 62
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
36469185 2022
11
Preceding polydipsia/polyuria, ataxia, and dysarthria in an adult with mixed histiocytosis (Erdheim Chester disease/Langerhans cell histiocytosis). 62
36197468 2022
12
Kingella kingae Osteomyelitis Should be Ruled out in Pediatric Langerhans Cell Histiocytosis. 62
36102720 2022
13
Classic cytomorphological features of Langerhans cell histiocytosis with osseous involvement. 62
36047490 2022
14
Diagnosis of Langerhans cell histiocytosis on cytological examination of cerebrospinal fluid: Report of the first case. 62
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
36335782 2022
16
Benign cephalic histiocytosis with extra-facial manifestations. 62
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
36471533 2022
18
Crystal-storing histiocytosis associated with monoclonal kappa light chain gammopathy. 62
35451182 2022
19
Strong Coexpression of Transcription Factors PU.1 and Oct-2 in Rosai-Dorfman Disease. 62
36239684 2022
20
68Ga-FAPI and 18F-FDG PET/CT Images of a Patient With Rosai-Dorfman Disease With Liver Involvement. 62
36342795 2022
21
A Review of Noninfectious Diseases Masquerading as Acute Mastoiditis. 62
34874762 2022
22
Incidence, prevalence and survival in patients with Langerhans cell histiocytosis: A national registry study from England, 2013-2019. 62
36122574 2022
23
The international dataset on the association between Langerhans Cell Histiocytosis and other malignancies. 62
36188132 2022
24
Biomodulatory therapy induces durable remissions in multi-system Langerhans cell histiocytosis. 62
35819881 2022
25
High-Resolution Computed Tomography of Cystic Lung Disease. 62
36252611 2022
26
Narrowing the Differential Diagnosis of Cystic Lesions in Smokers with Expiratory CT Acquisition Using the Cyst-Airway Communication Hypothesis. 62
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
36156506 2022
28
Langerhans Cell Histiocytosis Associated With Renal Cell Carcinoma: Is it an Indolent Phenomenon or a More Ominous Pathologic Process? 62
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
36362761 2022
30
Erdheim-Chester disease: look it in the eye. An orbital magnetic resonance imaging study. 62
35484665 2022
31
Airspace Enlargement with Fibrosis in a Young Heavy Smoker Mimicking Diffuse Cystic Lung Disease. 62
36422188 2022
32
Unexplained fever in children-Benefits and challenges of FDG-PET/CT. 62
36210785 2022
33
Imaging of Smoking and Vaping Related Diffuse Lung Injury. 62
36202480 2022
34
Solitary Involvement of the Liver: A Rare Manifestation of Langerhans Cell Histiocytosis. 62
36404611 2022
35
Rosai-Dorfman-Destombes Disease in the Pediatric Head and Neck. 62
36330598 2022
36
MUM1/IRF4 is Highly Expressed in Dermatopathic Lymphadenopathy: Potential Utility in Diagnosis and Differential Diagnosis. 62
35877199 2022
37
[Rosai-Dorfman-Destombes disease with a misleading laryngeal presentation]. 62
36038429 2022
38
Eye movement abnormalities in neurodegenerative langerhans cell histiocytosis. 62
35760933 2022
39
Immune-mediated diseases involving central and peripheral nervous systems. 62
36366904 2022
40
Clinical response to dabrafenib and chemotherapy in clonally-related histiocytosis and acute lymphoblastic leukemia. 62
36384252 2022
41
Curative Pericardiectomy in Interpheron-Resistant Severe Pericardial Erdheim-Chester Disease. 62
36444187 2022
42
Congenital solitary reticulohistiocytosis (Hashimoto - Pritzker). 62
36153176 2022
43
Rare presentation of multiple thromboses in Erdheim-Chester disease: a case-based review. 62
35687294 2022
44
Does Fractal Analysis Have a Role in Diagnosis of Langerhans Cell Histiocytosis? 62
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
35648675 2022
46
A rare case of Haemophagocytic Lymphohistiocytosis preceding a diagnosis of Langerhans cell histiocytosis. 62
35652532 2022
47
Open and Endoscopic Endonasal Biopsies for Langerhans Cell Histiocytosis of the Hypothalamus: 2-Dimensional Operative Video. 62
36113147 2022
48
VE1 immunohistochemistry to determine BRAFV600E mutation in Langerhans-cell histiocytosis. 62
36192351 2022
49
Whole-body magnetic resonance imaging for staging Langerhans cell histiocytosis in children and young adults. 62
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
36349275 2022

Variations for Histiocytosis

Expression for Histiocytosis

Search GEO for disease gene expression data for Histiocytosis.

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
Show member pathways
13.69 TNFRSF8 TNF SERPINA3 S100B PTPRC PECAM1
2
Show member pathways
13.55 TNFRSF8 TNF NRAS MAP2K1 CCR6 BRAF
3
Show member pathways
12.77 TNF NRAS MAP2K1 BRAF ARAF
4
Show member pathways
12.75 TNF NRAS MAP2K1 BRAF ARAF
5
Show member pathways
12.74 TNF NRAS MAP2K1 BRAF ARAF
6 12.73 ALK BRAF MAP2K1 NRAS S100B
7
Show member pathways
12.59 NRAS MAP2K1 BRAF ARAF ALK
8
Show member pathways
12.54 TNF NRAS MAP2K1 BRAF ARAF
9
Show member pathways
12.39 NRAS MAP2K1 BRAF ARAF
10
Show member pathways
12.37 NRAS MAP2K1 BRAF ARAF
11
Show member pathways
12.28 NRAS MAP2K1 BRAF ARAF ALK
12 12.24 TNF NRAS MAP2K1 BRAF ARAF
13
Show member pathways
12.14 NRAS MAP2K1 BRAF ARAF
14
Show member pathways
12.13 NRAS MAP2K1 BRAF ARAF
15
Show member pathways
12.06 ARAF BRAF MAP2K1 NRAS
16
Show member pathways
12.02 NRAS MAP2K1 BRAF ARAF
17
Show member pathways
11.99 NRAS MAP2K1 BRAF ARAF
18 11.86 TNF PTPRC CD1C CD163
19 11.83 NRAS MAP2K1 BRAF ARAF
20 11.81 MAP2K1 BRAF ARAF
21 11.68 MAP2K1 BRAF ARAF
22 11.64 TNF PTPRC CD101 CCR6
23
Show member pathways
11.6 ARAF BRAF MAP2K1 NRAS
24
Show member pathways
11.55 NRAS MAP2K1 BRAF
25
Show member pathways
11.54 NRAS MAP2K1 BRAF
26 11.19 NRAS MAP2K1 BRAF ARAF
27 11.16 TNF NRAS MAP2K1
28 11.06 NRAS MAP2K1 BRAF ARAF
29 10.97 TNF PTPRC CD207 CD1C CD1A CD163
30 10.93 NRAS MAP2K1 BRAF ARAF ALK
31 10.59 MAP2K1 BRAF ALK

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

Molecular functions related to Histiocytosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 exogenous lipid antigen binding GO:0030884 9.26 CD1C CD1A
2 endogenous lipid antigen binding GO:0030883 8.92 CD1C CD1A

Sources for Histiocytosis

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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