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MCID: HST010
MIFTS: 59

Histiocytosis

Categories: Respiratory diseases, Bone diseases, Blood diseases, Rare diseases, Immune diseases, Cancer 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
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
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 eosinophilic granuloma. 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 Etoposide and Doxorubicin have been mentioned in the context of this disorder. Affiliated tissues include bone, t cells and thyroid, and related phenotypes are proptosis and diabetes insipidus

Wikipedia : 76 In medicine, histiocytosis is an excessive number of histiocytes (tissue macrophages), and the term is... 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 373)
# Related Disease Score Top Affiliating Genes
1 alk+ histiocytosis 33.8 CD1A CD207 FSCN1
2 eosinophilic granuloma 33.7 BRAF CD1A
3 erdheim-chester disease 33.1 BRAF SERPINA3
4 juvenile xanthogranuloma 32.8 BRAF FSCN1 S100B
5 malignant histiocytosis 32.7 CSF1R SERPINA3 TNFRSF8
6 langerhans cell sarcoma 32.3 CD163 CD1A CD207
7 non-langerhans-cell histiocytosis 32.1 BRAF CD163 CD207 IL1RN SMPD1
8 langerhans cell histiocytosis 31.8 BRAF CD101 CD163 CD1A CD207 CSF2
9 dendritic cell tumor 31.3 CD1A CD207 FSCN1 PTPRC S100B
10 rosai-dorfman disease 31.2 CD163 FSCN1 MAP2K1 S100B SLC29A3 STAB1
11 histiocytoma 29.6 CD163 PTPRC SERPINA3
12 interdigitating dendritic cell sarcoma 29.6 CD163 PTPRC
13 malignant fibroxanthoma 29.6 PTPRC S100B SERPINA3
14 fibrous histiocytoma 29.2 CD163 S100B SERPINA3
15 anaplastic large cell lymphoma 29.1 CSF1R PTPRC TNFRSF8
16 reticulohistiocytic granuloma 29.1 CD163 CD207 FSCN1 PTPRC SERPINA3 TNFRSF8
17 lymphoma, non-hodgkin, familial 28.0 BRAF CSF2 ITGAX PTPRC TNFRSF8
18 histiocytosis-lymphadenopathy plus syndrome 12.5
19 histiocytosis, progressive mucinous 12.3
20 benign cephalic histiocytosis 12.3
21 generalized eruptive histiocytosis 12.3
22 indeterminate cell histiocytosis 12.2
23 progressive nodular histiocytosis 12.2
24 adult pulmonary langerhans cell histiocytosis 12.2
25 hashimoto-pritzker syndrome 12.0
26 sea-blue histiocyte disease 11.9
27 letterer-siwe disease 11.9
28 histiocytosis, familial lipochrome 11.8
29 gaucher's disease 11.4
30 niemann-pick disease 11.4
31 hemophagocytic lymphohistiocytosis 11.1
32 necrobiotic xanthogranuloma 11.1
33 papular xanthoma 11.1
34 interstitial lung disease 11.1
35 omenn syndrome 10.8
36 polymorphic reticulosis 10.8
37 primary cutaneous anaplastic large cell lymphoma 10.8
38 dentin dysplasia, type i 10.8
39 dysosteosclerosis 10.8
40 infantile myofibromatosis 10.8
41 myopericytoma 10.5 BRAF S100B
42 malignant giant cell tumor of soft parts 10.5 PTPRC S100B
43 syringocystadenoma papilliferum 10.5 BRAF S100B
44 cavernous sinus meningioma 10.4 CD163 CD207
45 nodular tenosynovitis 10.4 PTPRC SERPINA3
46 lymphoma 10.4
47 xanthoma disseminatum 10.4
48 chronic monocytic leukemia 10.3 ITGAX PTPRC
49 tenosynovial giant cell tumor 10.3 CSF1R SERPINA3
50 leukemia 10.2

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

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 SLC29A3 PTPRC
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 SLC29A3 CD163
8 Increased shRNA abundance (Z-score > 2) GR00366-A-149 9.89 BRAF SLC29A3 CD163 PTPRC
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 SLC29A3 CD163
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 cellular MP:0005384 9.85 CSF1R CSF2 FSCN1 IL1RN MAP2K1 PTPRC
2 hematopoietic system MP:0005397 9.73 BRAF CD101 CD163 CD207 CSF1R CSF2
3 immune system MP:0005387 9.44 BRAF CD101 CD163 CD207 CSF1R CSF2
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 166)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Etoposide Approved Phase 3,Phase 2,Not Applicable 33419-42-0 36462
2
Doxorubicin Approved, Investigational Phase 3,Phase 2 23214-92-8 31703
3
Hydrocortisone Approved, Vet_approved Phase 3,Phase 2 50-23-7 5754
4
Vincristine Approved, Investigational Phase 2, Phase 3 2068-78-2, 57-22-7 5978
5
Methotrexate Approved Phase 3,Phase 2,Not Applicable 1959-05-2, 59-05-2 126941
6
Miconazole Approved, Investigational, Vet_approved Phase 3,Phase 2,Phase 1,Not Applicable 22916-47-8 4189
7
Cyclophosphamide Approved, Investigational Phase 3,Phase 2,Phase 1,Not Applicable 50-18-0, 6055-19-2 2907
8
Dexamethasone Approved, Investigational, Vet_approved Phase 3,Phase 2,Not Applicable 50-02-2 5743
9
Busulfan Approved, Investigational Phase 3,Phase 2,Phase 1,Not Applicable,Early Phase 1 55-98-1 2478
10
Prednisolone Approved, Vet_approved Phase 3,Phase 2,Phase 1,Not Applicable 50-24-8 5755
11
Mycophenolic acid Approved Phase 3,Phase 2,Phase 1 24280-93-1 446541
12
Mycophenolate mofetil Approved, Investigational Phase 3,Phase 2,Phase 1 128794-94-5 5281078
13
Methylprednisolone Approved, Vet_approved Phase 3,Phase 2,Phase 1,Not Applicable 83-43-2 6741
14
Prednisone Approved, Vet_approved Phase 3,Phase 2,Not Applicable 53-03-2 5865
15
Vinblastine Approved Phase 3,Phase 2,Not Applicable 865-21-4 13342 241903
16
Miglustat Approved Phase 3,Phase 2,Phase 1 72599-27-0 51634
17
Cytarabine Approved, Investigational Phase 2, Phase 3,Phase 3 147-94-4 6253
18
Indomethacin Approved, Investigational Phase 2, Phase 3 53-86-1 3715
19
Cladribine Approved, Investigational Phase 2, Phase 3 4291-63-8 20279
20
Pegaspargase Approved, Investigational Phase 3 130167-69-0
21
leucovorin Approved, Nutraceutical Phase 3,Phase 2,Not Applicable 58-05-9 143 6006
22
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3,Phase 2,Not Applicable 59-30-3 6037
23
Butyric Acid Experimental, Investigational Phase 3,Phase 2 107-92-6 264
24
Doxil Approved June 1999 Phase 3,Phase 2 31703
25
1-Deoxynojirimycin Experimental Phase 3,Phase 2 19130-96-2 1374
26 Prednisolone acetate Phase 3,Phase 2,Phase 1,Not Applicable
27 Dexamethasone acetate Phase 3,Phase 2,Not Applicable 1177-87-3
28 Alkylating Agents Phase 3,Phase 2,Phase 1,Not Applicable
29 glucocorticoids Phase 3,Phase 2,Phase 1,Not Applicable
30 Methylprednisolone acetate Phase 3,Phase 2,Phase 1,Not Applicable
31 Methylprednisolone Hemisuccinate Phase 3,Phase 2,Phase 1,Not Applicable
32 Folic Acid Antagonists Phase 3,Phase 2,Not Applicable
33 Gastrointestinal Agents Phase 3,Phase 2,Phase 1,Not Applicable
34 Neuroprotective Agents Phase 3,Phase 2,Phase 1,Not Applicable
35 Cortisol succinate Phase 3,Phase 2
36 Cyclosporins Phase 3,Phase 2,Phase 1,Not Applicable
37 Topoisomerase Inhibitors Phase 3,Phase 2,Not Applicable
38 Hormone Antagonists Phase 3,Phase 2,Phase 1,Not Applicable
39 Dermatologic Agents Phase 3,Phase 2,Phase 1,Not Applicable
40 Nucleic Acid Synthesis Inhibitors Phase 3,Phase 2,Not Applicable
41 Hormones Phase 3,Phase 2,Phase 1,Not Applicable
42 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3,Phase 2,Phase 1,Not Applicable
43 Hydrocortisone 17-butyrate 21-propionate Phase 3,Phase 2
44 Hydrocortisone acetate Phase 3,Phase 2
45 Hydrocortisone-17-butyrate Phase 3,Phase 2
46 Peripheral Nervous System Agents Phase 3,Phase 2,Phase 1,Not Applicable
47 Anti-Bacterial Agents Phase 3,Phase 2,Phase 1
48 Vitamin B Complex Phase 3,Phase 2,Not Applicable
49 Antibiotics, Antitubercular Phase 3,Phase 2,Phase 1
50 Etoposide phosphate Phase 3,Phase 2,Not Applicable

Interventional clinical trials:

(show top 50) (show all 133)
# Name Status NCT ID Phase Drugs
1 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
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 Treatment Protocol for Hemophagocytic Lymphohistiocytosis 2004 Completed NCT00426101 Phase 3 Dexamethasone;Etoposide;Cyclosporin
4 Application of Miglustat in Patients With Niemann-Pick Type C Completed NCT01760564 Phase 3 Miglustat
5 Stem Cell Transplant for Inborn Errors of Metabolism Completed NCT00176904 Phase 2, Phase 3 Busulfan, Cyclophosphamide, Antithymocyte Globulin
6 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
7 Vinblastine/Prednisone Versus Single Therapy With Cytarabine for Langerhans Cell Histiocytosis (LCH) Recruiting NCT02670707 Phase 3 Cytarabine;Vinblastine/prednisone
8 DEP-Ru Regimen as a Salvage Therapy for HLH Recruiting NCT03533790 Phase 3 DEP-Ru
9 L-DEP as an Initial Treatment for EBV-HLH Recruiting NCT02912702 Phase 3 Pegaspargase;doxorubicin hydrochloride liposome injection;etoposide;methylprednisolone;Etoposide;dexamethasone
10 Splenectomy as a Treatment for Patient With Relapsed Haemophagocytic Lymphohistiocytosis of Unknown Etiology Recruiting NCT02862054 Phase 2, Phase 3
11 L-DEP Regimen as a Salvage Therapy for Refractory Epstein Barr Virus-induced Hemophagocytic Lymphohistiocytosis Recruiting NCT02631109 Phase 3 Pegaspargase;doxorubicin;etoposide;methylprednisolone
12 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
13 Efficacy, Safety, Pharmacodynamic, and Pharmacokinetics Study of Olipudase Alfa in Patients With Acid Sphingomyelinase Deficiency Recruiting NCT02004691 Phase 2, Phase 3 placebo (saline);GZ402665
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 Study to Assess the Efficacy and Safety of Emapalumab in Primary Haemophagocytic Lymphohistiocytosis Not yet recruiting NCT03312751 Phase 3 Emapalumab
17 T-Cell Depletion and Stem Cell Transplant for Immune Deficiencies and Histiocytic Disorders Terminated NCT00176826 Phase 2, Phase 3 Myeloablative conditioning regimen
18 Umbilical Cord Blood and Placental Blood Transplantation in Treating Patients With Hematologic Cancer or Aplastic Anemia Unknown status NCT00008164 Phase 2
19 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
20 Trial of DA-EPOCH Regimen for NHL With HLH Unknown status NCT01818908 Phase 2 DA-EPOCH
21 Treatment of Resistant Langerhans Cell Histiocytosis With ENBREL Completed NCT00048373 Phase 2 Etanercept
22 A Study to Test the Ability of and Safety of GSK2110183 in Treating Langerhans Cell Histiocytosis Completed NCT01395004 Phase 2 GSK2110183
23 Study of Sequential Administration of Oral 6-Thioguanine After Methotrexate in Patients With LCH Completed NCT00588536 Phase 2 Methotrexate;6-Thioguanine;Leucovorin Calcium
24 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
25 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)
26 Deferasirox in Treating Iron Overload Caused By Blood Transfusions in Patients With Hematologic Malignancies Completed NCT01273766 Phase 2 deferasirox
27 Hybrid Immunotherapy for Hemophagocytic LymphoHistiocytosis Completed NCT01104025 Phase 2 ATG, rabbit;Etoposide;Methotrexate;hydrocortisone;Dexamethasone
28 A Study of Vemurafenib in Participants With BRAF V600 Mutation-Positive Cancers Completed NCT01524978 Phase 2 cetuximab;vemurafenib;vemurafenib
29 Phase 1/2 Study of Vorinostat Therapy in Niemann-Pick Disease, Type C1 Completed NCT02124083 Phase 1, Phase 2 Vorinostat
30 Reduced Intensity Conditioning for Hemophagocytic Syndromes or Selected Primary Immune Deficiencies (BMT CTN 1204) Completed NCT01998633 Phase 2
31 Biomarker Validation for Niemann-Pick Disease, Type C: Safety and Efficacy of N-Acetyl Cysteine Completed NCT00975689 Phase 1, Phase 2 N-Acetyl Cysteine
32 Unrelated Hematopoietic Stem Cell Transplantation(HSCT) for Genetic Diseases of Blood Cells Completed NCT00730314 Phase 1, Phase 2
33 Miglustat in Niemann-Pick Type C Disease Completed NCT00517153 Phase 2 miglustat
34 Phase II Study of Clofarabine in Patients With Recurrent or Refractory Langerhans Cell Histiocytosis Recruiting NCT02425904 Phase 2 Clofarabine
35 Methotrexate and Cytosine in Adult Langerhans Cell Histiocytosis Recruiting NCT02389400 Phase 2 methotrexate
36 Denosumab for the Treatment of Adult LCH Recruiting NCT03270020 Phase 2 Denosumab 70 MG/ML [Xgeva]
37 Single-agent Cobimetinib for Adults With Histiocytic Disorders Recruiting NCT02649972 Phase 2 Cobimetinib
38 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;Vemurafenib
39 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
40 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
41 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
42 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
43 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
44 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
45 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
46 Ensartinib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With ALK or ROS1 Genomic Alterations (A Pediatric MATCH Treatment Trial) Recruiting NCT03213652 Phase 2 Ensartinib
47 Treatment of Familiar Lymphohistiocytosis Recruiting NCT02472054 Phase 1, Phase 2 Alemtuzumab;Methyl Prednisolone (MP);Cyclosporin A (CSA)
48 Alemtuzumab or Tocilizumab in Combination With Etoposide and Dexamethasone for the Treatment of Adult Patients With Hemophagocytic Lymphohistiocytosis Recruiting NCT02385110 Phase 2 Alemtuzumab;Etoposide;Dexamethasone;Methotrexate;Tocilizumab
49 Tocilizumab and Hemophagocytic Lymphohistiocytosis (HLH) Recruiting NCT02007239 Phase 2 tocilizumab
50 CD34+ (Malignant) Stem Cell Selection for Patients Receiving Allogenic Stem Cell Transplant Recruiting NCT02061800 Phase 1, Phase 2 Thiotepa;Cyclophosphamide;Alemtuzumab;Tacrolimus;Melphalan;Busulfan;Fludarabine;Methylprednisolone

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
Sources

Anatomical Context for Histiocytosis

About this section

MalaCards organs/tissues related to Histiocytosis:

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

Publications for Histiocytosis

About this section

Articles related to Histiocytosis:

(show top 50) (show all 1835)
# Title Authors Year
1
Reconstruction of a severe mandibular pathological fracture caused by Langerhans cell histiocytosis using a free fibula osteocutaneous flap:a case report. ( 29372175 )
2018
2
An update on the treatment of pediatric-onset Langerhans cell histiocytosis through pharmacotherapy. ( 29355432 )
2018
3
The use of BRAF V600E mutation-specific immunohistochemistry in pediatric Langerhans cell histiocytosis. ( 28219109 )
2018
4
Alemtuzumab-based therapy for Secondary Malignant Histiocytosis arising from Pre-B-ALL. ( 29264111 )
2018
5
Involvement of M1/M2 macrophages in the pathomechanisms of intralymphatic histiocytosis associated with rheumatoid arthritis. ( 29905385 )
2018
6
A Rare Case of Erdheim-Chester Disease (Non-Langerhans Cell Histiocytosis) with Concurrent Langerhans Cell Histiocytosis: A Diagnostic and Therapeutic Challenge. ( 29888013 )
2018
7
Crystal-storing Histiocytosis in the Stomach: A Clue to Subtle Hematolymphoid Malignancies. ( 29878935 )
2018
8
Generalized eruptive histiocytosis diagnosed in light of dermoscopic findings. ( 29243820 )
2018
9
Langerhans cell histiocytosis mimicking molluscum contagiosum: A case series. ( 29528195 )
2018
10
Oral manifestation of Langerhans cell histiocytosis: a case report. ( 29884166 )
2018
11
Reflectance Confocal Microscopy for the Diagnosis of Langerhans Cell Histiocytosis. ( 29341062 )
2018
12
A case report of orbital Langerhans cell histiocytosis presenting as a orbital cellulitis. ( 29643005 )
2018
13
Classic Hodgkin lymphoma with osseous involvement mimicking Langerhans cell histiocytosis in a child. ( 29307624 )
2018
14
Rosai-Dorfman Disease: Rare Pulmonary Involvement Mimicking Pulmonary Langerhans Cell Histiocytosis and Review of the Literature. ( 29850355 )
2018
15
Outcome After Radiation Therapy for Langerhans Cell Histiocytosis Is Dependent on Site of Involvement. ( 29413280 )
2018
16
Bone metabolism in Langerhans cell histiocytosis. ( 29967185 )
2018
17
Benign cephalic histiocytosis: A case with infiltration of CD1a-positive langerin-negative cells. ( 29430698 )
2018
18
Crystal-Storing Histiocytosis in Bone Marrow: A Clinicopathologic Study of Eight Cases and Review of the Literature. ( 29385417 )
2018
19
Langerhans cell histiocytosis of the orbit: A study of eight cases. ( 29930447 )
2018
20
National Jewish Health Classical Pulmonary Radiology Case Reports: Imaging Features of Langerhans Cell Histiocytosis. ( 29878226 )
2018
21
Radiotherapy for Langerhans cell histiocytosis with paraplegia: A rare oncologic emergency case report in infancy and literature review. ( 29907475 )
2018
22
Congenital Langerhans cell histiocytosis presenting in a 27-week-gestation neonate. ( 29436047 )
2018
23
MRI features in solitary cerebral Langerhans cell histiocytosis : a case report and review of literature. ( 29929024 )
2018
24
Langerhans cell histiocytosis in an 18-month-old child presenting as periorbital cellulitis. ( 29755272 )
2018
25
Langerhans cell histiocytosis of bone in an adult: A case report. ( 29904462 )
2018
26
Intraoperative MRI-guided biopsy in the diagnosis of suprasellar Langerhans Cell Histiocytosis. ( 29317365 )
2018
27
Rare childhood hybrid histiocytosis of the central nervous system-diagnosed by stereotactic brain biopsy with marked treatment response to clofarabine. ( 29872899 )
2018
28
Immune Thrombocytopenia in a Child With Refractory Langerhans Cell Histiocytosis Following Cladribine Containing Therapy. ( 29889804 )
2018
29
Pulmonary Langerhans cell histiocytosis, acute myeloid leukemia, and myelofibrosis in a large family and review of the literature. ( 29428448 )
2018
30
Pulmonary Langerhans Cell Histiocytosis(PLCH) masquerading as Atypical Mycobacterial infection. ( 29942736 )
2018
31
Indeterminate cell histiocytosis in a Chinese patient with progressive and extensive nodular lesions and mixed indeterminate cell and macrophage-monocyte lineage. ( 29469188 )
2018
32
Histiocytosis - cutaneous manifestations of hematopoietic neoplasm and non-neoplastic histiocytic proliferations. ( 29341328 )
2018
33
Langerhans Cell Histiocytosis of the Adult Cervical Spine: A Case Report and Literature Review. ( 29890538 )
2018
34
Crystal-Storing Histiocytosis with Plasma Cell Neoplasm in the Setting of Chronic Carbamazepine Exposure. ( 29874901 )
2018
35
Localised Langerhans cell histiocytosis of the hypothalamic-pituitary region: case report and literature review. ( 29858859 )
2018
36
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
37
Case of S100-positive benign cephalic histiocytosis involving monocyte/macrophage lineage marker expression. ( 29766556 )
2018
38
RAF/MEK/extracellular signal-related kinase pathway suppresses dendritic cell migration and traps dendritic cells in Langerhans cell histiocytosis lesions. ( 29263218 )
2018
39
A unique manifestation of Langerhans cell histiocytosis: Diagnostic and therapeutic considerations of atypical cases. ( 29940687 )
2018
40
Isolated Langerhans Cell Histiocytosis of Orbit: A Case Report and Review of the Literature. ( 29850325 )
2018
41
Juvenile Xanthogranuloma in a Pediatric Patient with Langerhans Cell Histiocytosis. ( 29765943 )
2018
42
Cutaneous manifestations of paediatric Erdheim-Chester disease, a histiocytosis of the 'L' group. ( 29357606 )
2018
43
A case of adult onset folliculocentric Langerhans cell histiocytosis. ( 29924409 )
2018
44
Successful management of multiple-systemic Langerhans cell histiocytosis involving endocrine organs in an adult: A case report and review of literature. ( 29952977 )
2018
45
Cutaneous Langerhans cell histiocytosis with gastrointestinal involvement treated with dabrafenib. ( 29379859 )
2018
46
A potentially important role for T cells and regulatory T cells in Langerhans cell histiocytosis. ( 29928999 )
2018
47
Disseminated juvenile xanthogranuloma occurring after treatment of Langerhans cell histiocytosis: a case report. ( 29209883 )
2017
48
Adult Langerhans cell histiocytosis with pulmonary and colorectoanal involvement: a case report. ( 28942734 )
2017
49
Congenital Self Healing Langerhans Cell Histiocytosis. ( 28929464 )
2017
50
Langerhans cell histiocytosis in a patient with hairy cell leukemia: a tale of divergence. ( 28302693 )
2017
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Variations for Histiocytosis

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Expression for Histiocytosis

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Search GEO for disease gene expression data for Histiocytosis.
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Pathways for Histiocytosis

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Pathways related to Histiocytosis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Innate Immune System 66
Show member pathways
 13.4 BRAF CD101 CD1A CD207 CSF1R CSF2
2
Cytokine Signaling in Immune system 66
Show member pathways
 12.87 BRAF CSF1R CSF2 FSCN1 IL1RN ITGAX
3
Hematopoietic Stem Cell Differentiation Pathways and Lineage-specific Markers 65
11.62 CD163 CSF2 ITGAX PTPRC
4
Hematopoietic cell lineage 37
11.48 CD1A CSF1R CSF2
5
Dendritic Cells Developmental Lineage Pathway 65
11.04 CD163 CD1A CD207 CSF1R CSF2 ITGAX
6
Serotonin Receptor 4/6/7 and NR3C Signaling 1
10.65 BRAF MAP2K1
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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 9.44 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 face development GO:0060324 9.37 BRAF MAP2K1
2 cytokine-mediated signaling pathway GO:0019221 9.35 CSF1R CSF2 FSCN1 IL1RN ITGAX
3 monocyte differentiation GO:0030224 9.32 CSF1R CSF2
4 positive regulation of podosome assembly GO:0071803 9.26 CSF2 FSCN1
5 regulation of axon regeneration GO:0048679 9.16 BRAF MAP2K1
6 inflammatory response GO:0006954 9.1 CD163 CSF1R IL1RN SERPINA3 STAB1 TNFRSF8

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

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 9.55 BRAF CD163 CD1A CD207 CSF1R CSF2
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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