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MCID: HST010
MIFTS: 61

Histiocytosis

Categories: Blood diseases, Bone diseases, Cancer diseases, Immune diseases, Rare diseases, Respiratory diseases
Genes Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Histiocytosis

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MalaCards integrated aliases for Histiocytosis:

Name: Histiocytosis 12 55 44 15 73
Chronic Multifocal Langerhans Cell Histiocytosis 59
Multifocal Eosinophilic Granuloma 59
Hand Schuller Christian Disease 12
Hand-Schüller-Christian Disease 59
Histiocytosis, Langerhans-Cell 73
Handsch�llerchristian Disease 76
Chronic Histiocytosis X 12
Histiocytic Syndrome 73

Classifications:

MalaCards categories:
Global: Rare diseases Cancer diseases
Anatomical: Respiratory diseases Bone diseases Blood diseases Immune diseases
See all MalaCards categories (disease lists)
ICD10: 34
Orphanet: 59  
Rare respiratory diseases
Rare systemic and rhumatological diseases
Rare haematological diseases


External Ids:

Disease Ontology 12 DOID:3405
MeSH 44 D015614
NCIt 50 C3106
SNOMED-CT 68 60657004 65396000
Orphanet 59 ORPHA99873
ICD10 via Orphanet 34 C96.5
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Summaries for Histiocytosis

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Disease Ontology : 12 A lymphatic system disease that is characterized by an excessive number of histiocytes.

MalaCards based summary : Histiocytosis, also known as chronic multifocal langerhans cell histiocytosis, is related to alk+ histiocytosis and non-langerhans-cell histiocytosis. 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 Cytokine Signaling in Immune system. The drugs Dexamethasone acetate and leucovorin have been mentioned in the context of this disorder. Affiliated tissues include bone, lung and thyroid, and related phenotypes are hepatomegaly and diabetes insipidus

Wikipedia : 76 Hand�??Schüller�??Christian disease is associated with multifocal Langerhans cell... more...

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Related Diseases for Histiocytosis

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Diseases in the Histiocytosis family:

Malignant Histiocytosis

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

(show top 50) (show all 437)
# Related Disease Score Top Affiliating Genes
1 alk+ histiocytosis 33.5 CD1A CD207
2 non-langerhans-cell histiocytosis 33.5 BRAF CD163 CD207 IL1RN
3 malignant histiocytosis 33.4 CSF1R SERPINA3 TNFRSF8
4 langerhans cell histiocytosis 33.2 BRAF CD101 CD163 CD1A CD207 CSF2
5 eosinophilic granuloma 33.2 BRAF CD1A
6 erdheim-chester disease 33.2 BRAF SERPINA3
7 juvenile xanthogranuloma 32.7 BRAF S100B
8 rosai-dorfman disease 32.4 CD163 MAP2K1 S100B SLC29A3 STAB1
9 langerhans cell sarcoma 32.2 CD163 CD1A CD207
10 dendritic cell tumor 31.9 CD1A CD207 PTPRC S100B
11 reticulohistiocytic granuloma 31.6 CD163 CD207 PTPRC SERPINA3 TNFRSF8
12 histiocytoma 29.9 CD163 PTPRC SERPINA3
13 malignant fibroxanthoma 29.7 PTPRC S100B SERPINA3
14 fibrous histiocytoma 29.6 CD163 S100B SERPINA3
15 interdigitating dendritic cell sarcoma 29.5 CD163 PTPRC
16 anaplastic large cell lymphoma 29.4 CSF1R PTPRC TNFRSF8
17 lymphoma, non-hodgkin, familial 29.4 BRAF CSF2 PTPRC TNFRSF8
18 histiocytosis-lymphadenopathy plus syndrome 12.7
19 histiocytosis, progressive mucinous 12.5
20 benign cephalic histiocytosis 12.5
21 generalized eruptive histiocytosis 12.4
22 indeterminate cell histiocytosis 12.4
23 progressive nodular histiocytosis 12.4
24 adult pulmonary langerhans cell histiocytosis 12.3
25 sea-blue histiocyte disease 12.2
26 hashimoto-pritzker syndrome 12.1
27 letterer-siwe disease 12.0
28 histiocytosis, familial lipochrome 12.0
29 gaucher's disease 11.6
30 niemann-pick disease 11.5
31 polymorphic reticulosis 11.3
32 hemophagocytic lymphohistiocytosis 11.3
33 necrobiotic xanthogranuloma 11.3
34 papular xanthoma 11.3
35 interstitial lung disease 11.3
36 dentin dysplasia, type i 10.9
37 dysosteosclerosis 10.9
38 omenn syndrome 10.9
39 infantile myofibromatosis 10.9
40 primary cutaneous anaplastic large cell lymphoma 10.9
41 lymphoma 10.5
42 xanthoma disseminatum 10.5
43 diabetes insipidus 10.4
44 leukemia 10.4
45 lipogranulomatosis 10.4
46 sarcoma 10.2
47 pneumothorax 10.2
48 myopericytoma 10.2 BRAF S100B
49 palladium allergic contact dermatitis 10.2 CSF2 IL17A
50 syringocystadenoma papilliferum 10.2 BRAF S100B

Graphical network of the top 20 diseases related to Histiocytosis:



Diseases related to Histiocytosis
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Symptoms & Phenotypes for Histiocytosis

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Human phenotypes related to Histiocytosis:

59 32 (show all 7)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hepatomegaly 59 32 occasional (7.5%) Occasional (29-5%) HP:0002240
2 diabetes insipidus 59 32 hallmark (90%) Very frequent (99-80%) HP:0000873
3 proptosis 59 32 hallmark (90%) Very frequent (99-80%) HP:0000520
4 osteolysis 59 32 hallmark (90%) Very frequent (99-80%) HP:0002797
5 papule 59 32 occasional (7.5%) Occasional (29-5%) HP:0200034
6 lymphadenopathy 59 32 occasional (7.5%) Occasional (29-5%) HP:0002716
7 pulmonary infiltrates 59 32 occasional (7.5%) Occasional (29-5%) HP:0002113

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

26 (show all 30)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-105 9.89 PTPRC SLC29A3
2 Increased shRNA abundance (Z-score > 2) GR00366-A-108 9.89 SLC29A3
3 Increased shRNA abundance (Z-score > 2) GR00366-A-110 9.89 BRAF
4 Increased shRNA abundance (Z-score > 2) GR00366-A-121 9.89 PTPRC
5 Increased shRNA abundance (Z-score > 2) GR00366-A-124 9.89 CD163
6 Increased shRNA abundance (Z-score > 2) GR00366-A-13 9.89 SLC29A3
7 Increased shRNA abundance (Z-score > 2) GR00366-A-137 9.89 CD163 SLC29A3
8 Increased shRNA abundance (Z-score > 2) GR00366-A-149 9.89 BRAF CD163 PTPRC SLC29A3
9 Increased shRNA abundance (Z-score > 2) GR00366-A-166 9.89 BRAF
10 Increased shRNA abundance (Z-score > 2) GR00366-A-168 9.89 SLC29A3
11 Increased shRNA abundance (Z-score > 2) GR00366-A-177 9.89 BRAF CD163
12 Increased shRNA abundance (Z-score > 2) GR00366-A-178 9.89 SLC29A3
13 Increased shRNA abundance (Z-score > 2) GR00366-A-194 9.89 BRAF
14 Increased shRNA abundance (Z-score > 2) GR00366-A-2 9.89 PTPRC
15 Increased shRNA abundance (Z-score > 2) GR00366-A-21 9.89 SLC29A3
16 Increased shRNA abundance (Z-score > 2) GR00366-A-214 9.89 PTPRC
17 Increased shRNA abundance (Z-score > 2) GR00366-A-29 9.89 BRAF
18 Increased shRNA abundance (Z-score > 2) GR00366-A-31 9.89 BRAF
19 Increased shRNA abundance (Z-score > 2) GR00366-A-32 9.89 BRAF
20 Increased shRNA abundance (Z-score > 2) GR00366-A-41 9.89 PTPRC
21 Increased shRNA abundance (Z-score > 2) GR00366-A-47 9.89 BRAF
22 Increased shRNA abundance (Z-score > 2) GR00366-A-49 9.89 SLC29A3
23 Increased shRNA abundance (Z-score > 2) GR00366-A-50 9.89 PTPRC
24 Increased shRNA abundance (Z-score > 2) GR00366-A-52 9.89 CD163
25 Increased shRNA abundance (Z-score > 2) GR00366-A-70 9.89 SLC29A3
26 Increased shRNA abundance (Z-score > 2) GR00366-A-74 9.89 CD163
27 Increased shRNA abundance (Z-score > 2) GR00366-A-8 9.89 SLC29A3
28 Increased shRNA abundance (Z-score > 2) GR00366-A-81 9.89 CD163 SLC29A3
29 Increased shRNA abundance (Z-score > 2) GR00366-A-83 9.89 CD163
30 Increased shRNA abundance (Z-score > 2) GR00366-A-9 9.89 CD163

MGI Mouse Phenotypes related to Histiocytosis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10 BRAF CD101 CD163 CD207 CSF1R CSF2
2 homeostasis/metabolism MP:0005376 9.85 BRAF CD163 CSF1R CSF2 IL17A IL1RN
3 immune system MP:0005387 9.7 BRAF CD101 CD163 CD207 CSF1R CSF2
4 respiratory system MP:0005388 9.17 BRAF CD163 CD207 CSF2 IL17A PTPRC
Sources

Drugs & Therapeutics for Histiocytosis

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

(show top 50) (show all 181)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 3,Phase 2,Not Applicable 1177-87-3
2
leucovorin Approved Phase 3,Phase 2,Not Applicable 58-05-9 6006 143
3
Hydrocortisone Approved, Vet_approved Phase 3,Phase 2 50-23-7 5754
4
Methotrexate Approved Phase 3,Phase 2,Not Applicable 59-05-2, 1959-05-2 126941
5
Busulfan Approved, Investigational Phase 3,Phase 2,Phase 1,Not Applicable,Early Phase 1 55-98-1 2478
6
Cyclophosphamide Approved, Investigational Phase 3,Phase 2,Phase 1,Not Applicable 6055-19-2, 50-18-0 2907
7
Mycophenolic acid Approved Phase 3,Phase 2,Phase 1 24280-93-1 446541
8
Miconazole Approved, Investigational, Vet_approved Phase 3,Phase 2,Phase 1,Not Applicable 22916-47-8 4189
9
Dexamethasone Approved, Investigational, Vet_approved Phase 3,Phase 2,Not Applicable 50-02-2 5743
10
Etoposide Approved Phase 3,Phase 2,Not Applicable 33419-42-0 36462
11
Hydrocortisone acetate Approved, Vet_approved Phase 3,Phase 2 50-03-3
12
Prednisone Approved, Vet_approved Phase 3,Phase 2,Not Applicable 53-03-2 5865
13
Methylprednisolone hemisuccinate Approved Phase 3,Phase 2,Phase 1,Not Applicable 2921-57-5
14
Prednisolone Approved, Vet_approved Phase 3,Phase 2,Phase 1,Not Applicable 50-24-8 5755
15
Methylprednisolone Approved, Vet_approved Phase 3,Phase 2,Phase 1,Not Applicable 83-43-2 6741
16
Prednisolone phosphate Approved, Vet_approved Phase 3,Phase 2,Phase 1,Not Applicable 302-25-0
17
Vincristine Approved, Investigational Phase 2, Phase 3 57-22-7, 2068-78-2 5978
18
Doxorubicin Approved, Investigational Phase 3,Phase 2 23214-92-8 31703
19
Vinblastine Approved Phase 3,Phase 2,Not Applicable 865-21-4 241903 13342
20
Miglustat Approved Phase 3,Phase 2,Phase 1 72599-27-0 51634
21
Cytarabine Approved, Investigational Phase 2, Phase 3,Phase 3 147-94-4 6253
22
Indomethacin Approved, Investigational Phase 2, Phase 3 53-86-1 3715
23
Cladribine Approved, Investigational Phase 2, Phase 3 4291-63-8 20279
24
Pegaspargase Approved, Investigational Phase 3 130167-69-0
25
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3,Phase 2,Not Applicable 59-30-3 6037
26
Prednisolone hemisuccinate Experimental Phase 3,Phase 2,Phase 1,Not Applicable 2920-86-7
27
Doxil Approved June 1999 Phase 3,Phase 2 31703
28
1-Deoxynojirimycin Experimental, Investigational Phase 3,Phase 2 19130-96-2 1374
29 Antitubercular Agents Phase 3,Phase 2,Phase 1
30 BB 1101 Phase 3,Phase 2,Not Applicable
31 Hydrocortisone 17-butyrate 21-propionate Phase 3,Phase 2
32 Nucleic Acid Synthesis Inhibitors Phase 3,Phase 2,Not Applicable
33 Antineoplastic Agents, Hormonal Phase 3,Phase 2,Phase 1,Not Applicable
34 Immunologic Factors Phase 3,Phase 2,Phase 1,Not Applicable
35 Antineoplastic Agents, Alkylating Phase 3,Phase 2,Phase 1,Not Applicable
36 Antirheumatic Agents Phase 3,Phase 2,Phase 1,Not Applicable
37 Peripheral Nervous System Agents Phase 3,Phase 2,Phase 1,Not Applicable
38 Antilymphocyte Serum Phase 3,Phase 2,Phase 1,Not Applicable
39 Hydrocortisone-17-butyrate Phase 3,Phase 2
40 Calcineurin Inhibitors Phase 3,Phase 2,Phase 1,Not Applicable
41 Anti-Inflammatory Agents Phase 3,Phase 2,Phase 1,Not Applicable
42 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3,Phase 2,Phase 1,Not Applicable
43 Antibiotics, Antitubercular Phase 3,Phase 2,Phase 1
44 Antifungal Agents Phase 3,Phase 2,Phase 1,Not Applicable
45 Hormones Phase 3,Phase 2,Phase 1,Not Applicable
46 Antiemetics Phase 3,Phase 2,Phase 1,Not Applicable
47 Vitamin B9 Phase 3,Phase 2,Not Applicable
48 Dermatologic Agents Phase 3,Phase 2,Phase 1,Not Applicable
49 Vitamin B Complex Phase 3,Phase 2,Not Applicable
50 Etoposide phosphate Phase 3,Phase 2,Not Applicable

Interventional clinical trials:

(show top 50) (show all 144)
# Name Status NCT ID Phase Drugs
1 Splenectomy as a Treatment for Patient With Relapsed Haemophagocytic Lymphohistiocytosis of Unknown Etiology Unknown status NCT02862054 Phase 2, Phase 3
2 Combination Chemotherapy Followed By Donor Stem Cell Transplant in Treating Patients With Hemophagocytic Lymphohistiocytosis Unknown status NCT00334672 Phase 3 busulfan;cyclophosphamide;cyclosporine;dexamethasone;etoposide;methotrexate;mycophenolate mofetil;therapeutic hydrocortisone
3 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
4 Treatment Protocol for Hemophagocytic Lymphohistiocytosis 2004 Completed NCT00426101 Phase 3 Dexamethasone;Etoposide;Cyclosporin
5 Application of Miglustat in Patients With Niemann-Pick Type C Completed NCT01760564 Phase 3 Miglustat
6 Stem Cell Transplant for Inborn Errors of Metabolism Completed NCT00176904 Phase 2, Phase 3 Busulfan, Cyclophosphamide, Antithymocyte Globulin
7 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
8 Vinblastine/Prednisone Versus Single Therapy With Cytarabine for Langerhans Cell Histiocytosis (LCH) Recruiting NCT02670707 Phase 3 Cytarabine;Vinblastine/prednisone
9 DEP-Ru Regimen as a Salvage Therapy for HLH Recruiting NCT03533790 Phase 3 DEP-Ru
10 Study to Assess the Efficacy and Safety of Emapalumab in Primary Haemophagocytic Lymphohistiocytosis Recruiting NCT03312751 Phase 3 Emapalumab
11 L-DEP as an Initial Treatment for EBV-HLH Recruiting NCT02912702 Phase 3 Pegaspargase;doxorubicin hydrochloride liposome injection;etoposide;methylprednisolone;Etoposide;dexamethasone
12 L-DEP Regimen as a Salvage Therapy for Refractory Epstein Barr Virus-induced Hemophagocytic Lymphohistiocytosis Recruiting NCT02631109 Phase 3 Pegaspargase;doxorubicin;etoposide;methylprednisolone
13 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
14 Arimoclomol Prospective Study in Patients Diagnosed With NiemannPick Disease Type C Active, not recruiting NCT02612129 Phase 2, Phase 3 arimoclomol;Placebo
15 Study of VTS-270 (2-hydroxypropyl-β-cyclodextrin) to Treat Niemann-Pick Type C1 (NPC1) Disease Active, not recruiting NCT02534844 Phase 2, Phase 3 VTS-270;Sham Procedure Control
16 Efficacy, Safety, Pharmacodynamic, and Pharmacokinetics Study of Olipudase Alfa in Patients With Acid Sphingomyelinase Deficiency Active, not recruiting NCT02004691 Phase 2, Phase 3 placebo (saline);GZ402665
17 Etoposide in the First-line Treatment of Adult EBV-HLH Not yet recruiting NCT03742115 Phase 3 Etoposide;Dexamethasone;Etoposide;IVIG
18 T-Cell Depletion and Stem Cell Transplant for Immune Deficiencies and Histiocytic Disorders Terminated NCT00176826 Phase 2, Phase 3 Myeloablative conditioning regimen
19 Umbilical Cord Blood and Placental Blood Transplantation in Treating Patients With Hematologic Cancer or Aplastic Anemia Unknown status NCT00008164 Phase 2
20 Umbilical Cord Blood for Stem Cell Transplantation in Treating Young Patients With Malignant or Nonmalignant Diseases Unknown status NCT00084695 Phase 2 busulfan;cyclophosphamide;fludarabine phosphate;melphalan;methylprednisolone
21 Trial of DA-EPOCH Regimen for NHL With HLH Unknown status NCT01818908 Phase 2 DA-EPOCH
22 Treatment of Resistant Langerhans Cell Histiocytosis With ENBREL Completed NCT00048373 Phase 2 Etanercept
23 Methotrexate and Cytosine in Adult Langerhans Cell Histiocytosis Completed NCT02389400 Phase 2 methotrexate
24 A Study to Test the Ability of and Safety of GSK2110183 in Treating Langerhans Cell Histiocytosis Completed NCT01395004 Phase 2 GSK2110183
25 Study of Sequential Administration of Oral 6-Thioguanine After Methotrexate in Patients With LCH Completed NCT00588536 Phase 2 Methotrexate;6-Thioguanine;Leucovorin Calcium
26 Dabrafenib and Trametinib in People With BRAF V600E Mutation Positive Lesions in Erdheim Chester Disease Completed NCT02281760 Phase 2 Dabrafenib Mesylate;Trametinib Dimethyl Sulfoxide
27 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
28 Stem Cell Transplant for Immunologic or Histiocytic Disorders Completed NCT00176865 Phase 2 Fludarabine;Melphalan;Anti-thymocyte globulin (ATG);Campath 1H;Cyclosporin A;Mycophenolate mofetil;Intravenous immunoglobulin (IVIG)
29 CD34+Selection for Partially Matched Family or Matched Unrelated Adult Donor Transplant Completed NCT01049854 Phase 2 Full Intensity with TBI;Full Intensity;Reduced Intensity;Reduced Intensity (Fanconi)
30 Deferasirox in Treating Iron Overload Caused By Blood Transfusions in Patients With Hematologic Malignancies Completed NCT01273766 Phase 2 deferasirox
31 Hybrid Immunotherapy for Hemophagocytic LymphoHistiocytosis Completed NCT01104025 Phase 2 ATG, rabbit;Etoposide;Methotrexate;hydrocortisone;Dexamethasone
32 A Study of Vemurafenib in Participants With BRAF V600 Mutation-Positive Cancers Completed NCT01524978 Phase 2 cetuximab;vemurafenib;vemurafenib
33 Phase 1/2 Study of Vorinostat Therapy in Niemann-Pick Disease, Type C1 Completed NCT02124083 Phase 1, Phase 2 Vorinostat
34 Reduced Intensity Conditioning for Hemophagocytic Syndromes or Selected Primary Immune Deficiencies (BMT CTN 1204) Completed NCT01998633 Phase 2
35 Biomarker Validation for Niemann-Pick Disease, Type C: Safety and Efficacy of N-Acetyl Cysteine Completed NCT00975689 Phase 1, Phase 2 N-Acetyl Cysteine
36 Unrelated Hematopoietic Stem Cell Transplantation(HSCT) for Genetic Diseases of Blood Cells Completed NCT00730314 Phase 1, Phase 2
37 Miglustat in Niemann-Pick Type C Disease Completed NCT00517153 Phase 2 miglustat
38 Study of Clofarabine in Patients With Recurrent or Refractory Langerhans Cell Histiocytosis and LCH-related Disorders Recruiting NCT02425904 Phase 2 Clofarabine
39 Denosumab for the Treatment of Adult LCH Recruiting NCT03270020 Phase 2 Denosumab 70 MG/ML [Xgeva]
40 A Combination of Vemurafenib, Cytarabine and 2-chlorodeoxyadenosine in Children With LCH and BRAF V600E Mutation Recruiting NCT03585686 Phase 2 Vemurafenib;Cytarabine;2-chlorodeoxyadenosine
41 Single-agent Cobimetinib for Adults With Histiocytic Disorders Recruiting NCT02649972 Phase 2 Cobimetinib
42 Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphomas, or Histiocytic Disorders (The Pediatric MATCH Screening Trial) Recruiting NCT03155620 Phase 2 Ensartinib;Erdafitinib;Larotrectinib;Olaparib;Palbociclib;PI3K/mTOR Inhibitor LY3023414;Selumetinib Sulfate;Tazemetostat;Ulixertinib;Vemurafenib
43 Olaparib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With Defects in DNA Damage Repair Genes (A Pediatric MATCH Treatment Trial) Recruiting NCT03233204 Phase 2 Olaparib
44 Erdafitinib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With FGFR Mutations (A Pediatric MATCH Treatment Trial) Recruiting NCT03210714 Phase 2 Erdafitinib
45 Vemurafenib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With BRAF V600 Mutations (A Pediatric MATCH Treatment Trial) Recruiting NCT03220035 Phase 2 Vemurafenib
46 Ulixertinib in Treating Patients With Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With MAPK Pathway Mutations (A Pediatric MATCH Treatment Trial) Recruiting NCT03698994 Phase 2 Ulixertinib
47 Palbociclib in Treating Patients With Relapsed or Refractory Rb Positive Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With Activating Alterations in Cell Cycle Genes (A Pediatric MATCH Treatment Trial) Recruiting NCT03526250 Phase 2 Palbociclib
48 Larotrectinib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With NTRK Fusions (A Pediatric MATCH Treatment Trial) Recruiting NCT03213704 Phase 2 Larotrectinib
49 PI3K/mTOR Inhibitor LY3023414 in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With TSC or PI3K/MTOR Mutations (A Pediatric MATCH Treatment Trial) Recruiting NCT03213678 Phase 2 PI3K/mTOR Inhibitor LY3023414
50 Selumetinib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With Activating MAPK Pathway Mutations (A Pediatric MATCH Treatment Trial) Recruiting NCT03213691 Phase 2 Selumetinib

Search NIH Clinical Center for Histiocytosis

Inferred drug relations via UMLS 73 / NDF-RT 51 :


Cochrane evidence based reviews: histiocytosis

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Genetic Tests for Histiocytosis

About this section
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Anatomical Context for Histiocytosis

About this section

MalaCards organs/tissues related to Histiocytosis:

41
Bone, Lung, Thyroid, Skin, Bone Marrow, Liver, T Cells
Sources

Publications for Histiocytosis

About this section

Articles related to Histiocytosis:

(show top 50) (show all 3235)
# Title Authors Year
1
Central diabetes insipidus in pediatric patients with Langerhans cell histiocytosis: Results from the JLSG-96/02 studies. ( 30207064 )
2019
2
Cladribine-related immunosuppression may have fostered graft-versus-host disease after lung transplant for pulmonary Langerhans cell histiocytosis. ( 30270491 )
2019
3
Bilateral pulmonary nodules and intravascular pulmonary histiocytosis: A rare presentation of hemophagocytic lymphohistiocytosis secondary to Epstein-Barr Virus infection. ( 30456165 )
2019
4
A novel fusion gene PLEKHA6-NTRK3 in langerhans cell histiocytosis. ( 30098202 )
2019
5
Reconstruction of a severe mandibular pathological fracture caused by Langerhans cell histiocytosis using a free fibula osteocutaneous flap:a case report. ( 29372175 )
2018
6
An update on the treatment of pediatric-onset Langerhans cell histiocytosis through pharmacotherapy. ( 29355432 )
2018
7
The use of BRAF V600E mutation-specific immunohistochemistry in pediatric Langerhans cell histiocytosis. ( 28219109 )
2018
8
Alemtuzumab-based therapy for Secondary Malignant Histiocytosis arising from Pre-B-ALL. ( 29264111 )
2018
9
Involvement of M1/M2 macrophages in the pathomechanisms of intralymphatic histiocytosis associated with rheumatoid arthritis. ( 29905385 )
2018
10
A Rare Case of Erdheim-Chester Disease (Non-Langerhans Cell Histiocytosis) with Concurrent Langerhans Cell Histiocytosis: A Diagnostic and Therapeutic Challenge. ( 29888013 )
2018
11
Crystal-storing Histiocytosis in the Stomach: A Clue to Subtle Hematolymphoid Malignancies. ( 29878935 )
2018
12
Generalized eruptive histiocytosis diagnosed in light of dermoscopic findings. ( 29243820 )
2018
13
Langerhans cell histiocytosis mimicking molluscum contagiosum: A case series. ( 29528195 )
2018
14
Oral manifestation of Langerhans cell histiocytosis: a case report. ( 29884166 )
2018
15
Reflectance Confocal Microscopy for the Diagnosis of Langerhans Cell Histiocytosis. ( 29341062 )
2018
16
A case report of orbital Langerhans cell histiocytosis presenting as a orbital cellulitis. ( 29643005 )
2018
17
Classic Hodgkin lymphoma with osseous involvement mimicking Langerhans cell histiocytosis in a child. ( 29307624 )
2018
18
Rosai-Dorfman Disease: Rare Pulmonary Involvement Mimicking Pulmonary Langerhans Cell Histiocytosis and Review of the Literature. ( 29850355 )
2018
19
Outcome After Radiation Therapy for Langerhans Cell Histiocytosis Is Dependent on Site of Involvement. ( 29413280 )
2018
20
Bone metabolism in Langerhans cell histiocytosis. ( 29967185 )
2018
21
Benign cephalic histiocytosis: A case with infiltration of CD1a-positive langerin-negative cells. ( 29430698 )
2018
22
Crystal-Storing Histiocytosis in Bone Marrow: A Clinicopathologic Study of Eight Cases and Review of the Literature. ( 29385417 )
2018
23
Langerhans cell histiocytosis of the orbit: A study of eight cases. ( 29930447 )
2018
24
National Jewish Health Classical Pulmonary Radiology Case Reports: Imaging Features of Langerhans Cell Histiocytosis. ( 29878226 )
2018
25
Radiotherapy for Langerhans cell histiocytosis with paraplegia: A rare oncologic emergency case report in infancy and literature review. ( 29907475 )
2018
26
Congenital Langerhans cell histiocytosis presenting in a 27-week-gestation neonate. ( 29436047 )
2018
27
MRI features in solitary cerebral Langerhans cell histiocytosis : a case report and review of literature. ( 29929024 )
2018
28
Langerhans cell histiocytosis in an 18-month-old child presenting as periorbital cellulitis. ( 29755272 )
2018
29
Langerhans cell histiocytosis of bone in an adult: A case report. ( 29904462 )
2018
30
Intraoperative MRI-guided biopsy in the diagnosis of suprasellar Langerhans Cell Histiocytosis. ( 29317365 )
2018
31
Rare childhood hybrid histiocytosis of the central nervous system-diagnosed by stereotactic brain biopsy with marked treatment response to clofarabine. ( 29872899 )
2018
32
Immune Thrombocytopenia in a Child With Refractory Langerhans Cell Histiocytosis Following Cladribine Containing Therapy. ( 29889804 )
2018
33
Pulmonary Langerhans cell histiocytosis, acute myeloid leukemia, and myelofibrosis in a large family and review of the literature. ( 29428448 )
2018
34
Pulmonary Langerhans Cell Histiocytosis(PLCH) masquerading as Atypical Mycobacterial infection. ( 29942736 )
2018
35
Indeterminate cell histiocytosis in a Chinese patient with progressive and extensive nodular lesions and mixed indeterminate cell and macrophage-monocyte lineage. ( 29469188 )
2018
36
Histiocytosis - cutaneous manifestations of hematopoietic neoplasm and non-neoplastic histiocytic proliferations. ( 29341328 )
2018
37
Langerhans Cell Histiocytosis of the Adult Cervical Spine: A Case Report and Literature Review. ( 29890538 )
2018
38
Crystal-Storing Histiocytosis with Plasma Cell Neoplasm in the Setting of Chronic Carbamazepine Exposure. ( 29874901 )
2018
39
Localised Langerhans cell histiocytosis of the hypothalamic-pituitary region: case report and literature review. ( 29858859 )
2018
40
Cutaneous squamous cell carcinoma, thyroid cancer and Langerhans cell histiocytosis in a patient with X-linked recessive Mendelian susceptibility to mycobacterial diseases with a nuclear factor-I_B essential modifier mutation. ( 29797522 )
2018
41
Case of S100-positive benign cephalic histiocytosis involving monocyte/macrophage lineage marker expression. ( 29766556 )
2018
42
RAF/MEK/extracellular signal-related kinase pathway suppresses dendritic cell migration and traps dendritic cells in Langerhans cell histiocytosis lesions. ( 29263218 )
2018
43
A unique manifestation of Langerhans cell histiocytosis: Diagnostic and therapeutic considerations of atypical cases. ( 29940687 )
2018
44
Isolated Langerhans Cell Histiocytosis of Orbit: A Case Report and Review of the Literature. ( 29850325 )
2018
45
Juvenile Xanthogranuloma in a Pediatric Patient with Langerhans Cell Histiocytosis. ( 29765943 )
2018
46
Cutaneous manifestations of paediatric Erdheim-Chester disease, a histiocytosis of the 'L' group. ( 29357606 )
2018
47
A case of adult onset folliculocentric Langerhans cell histiocytosis. ( 29924409 )
2018
48
Successful management of multiple-systemic Langerhans cell histiocytosis involving endocrine organs in an adult: A case report and review of literature. ( 29952977 )
2018
49
Cutaneous adult xanthogranuloma with a small portion of BRAFV600E mutated Langerhans cell histiocytosis populations: A case report and the review of published work. ( 30536719 )
2018
50
A Case of Unicentric Castleman Disease and Langerhans Cell Histiocytosis: Two Entities in One Lymph Node. ( 30549992 )
2018
Sources

Variations for Histiocytosis

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Expression for Histiocytosis

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Search GEO for disease gene expression data for Histiocytosis.
Sources

Pathways for Histiocytosis

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Pathways related to Histiocytosis according to GeneCards Suite gene sharing:

(show all 13)
# Super pathways Score Top Affiliating Genes
1
Innate Immune System 66
Show member pathways
 13.36 BRAF CD101 CD1A CD207 CSF1R CSF2
2
Cytokine Signaling in Immune system 66
Show member pathways
 12.91 BRAF CSF1R CSF2 IL17A IL1RN MAP2K1
3
NF-kappaB Signaling 4
12.27 IL17A IL1RN PTPRC TNFRSF8
4
JAK-STAT signaling pathway (KEGG) 37
Show member pathways
 12.07 CSF1R CSF2 MAP2K1 PTPRC
5
T cell receptor signaling pathway 37
Show member pathways
 12.03 CSF2 IL17A MAP2K1 PTPRC
6
Hematopoietic Stem Cell Differentiation Pathways and Lineage-specific Markers 65
11.72 CD163 CSF2 PTPRC
7
Jak-Stat Signaling Pathway (sino) 67
11.62 CSF1R MAP2K1 PTPRC
8
Hematopoietic cell lineage 37
11.6 CD1A CSF1R CSF2
9
Innate Lymphoid Cell Differentiation Pathways 65
11.45 CD101 IL17A PTPRC
10
Microglia Activation During Neuroinflammation: Microglia Polarization 65
11.31 CD163 CSF2 IL17A IL1RN
11
Dendritic Cells Developmental Lineage Pathway 65
10.97 CD163 CD1A CD207 CSF1R CSF2 PTPRC
12
Estrogen signaling pathway 1
10.89 BRAF MAP2K1
13
Serotonin Receptor 4/6/7 and NR3C Signaling 1
10.79 BRAF MAP2K1
Sources

GO Terms for Histiocytosis

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

# Name GO ID Score Top Affiliating Genes
1 plasma membrane GO:0005886 9.36 BRAF CD101 CD163 CD1A CD207 CSF1R

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

# Name GO ID Score Top Affiliating Genes
1 cytokine-mediated signaling pathway GO:0019221 9.67 CSF1R CSF2 IL17A IL1RN
2 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.62 BRAF CSF1R MAP2K1 PTPRC
3 thyroid gland development GO:0030878 9.48 BRAF MAP2K1
4 positive regulation of T cell mediated cytotoxicity GO:0001916 9.46 CD1A PTPRC
5 face development GO:0060324 9.43 BRAF MAP2K1
6 monocyte differentiation GO:0030224 9.4 CSF1R CSF2
7 regulation of axon regeneration GO:0048679 9.26 BRAF MAP2K1
8 peptidyl-tyrosine phosphorylation GO:0018108 9.26 BRAF CSF1R CSF2 MAP2K1
9 positive regulation of interleukin-23 production GO:0032747 9.16 CSF2 IL17A
10 inflammatory response GO:0006954 9.1 CD163 CSF1R IL17A IL1RN SERPINA3 STAB1

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

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 9.8 BRAF CD163 CD1A CD207 CSF1R CSF2
2 protein tyrosine kinase activity GO:0004713 8.92 BRAF CSF1R CSF2 MAP2K1
Sources

Sources for Histiocytosis

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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