MCID: HST010
MIFTS: 58

Histiocytosis malady

Categories: Respiratory diseases, Bone diseases, Blood diseases, Rare diseases, Immune diseases, Cancer diseases

Aliases & Classifications for Histiocytosis

Aliases & Descriptions for Histiocytosis:

Name: Histiocytosis 12 52 42 14 69
Chronic Multifocal Langerhans Cell Histiocytosis 56
Multifocal Eosinophilic Granuloma 56
Hand Schuller Christian Disease 12
Hand-Schüller-Christian Disease 56
Histiocytosis, Langerhans-Cell 69
Chronic Histiocytosis X 12
Histiocytic Syndrome 69

Classifications:



External Ids:

Disease Ontology 12 DOID:3405
MeSH 42 D015614
NCIt 47 C3106
Orphanet 56 ORPHA99873
ICD10 via Orphanet 34 C96.5
UMLS 69 C0019618

Summaries for Histiocytosis

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 juvenile xanthogranuloma and histiocytosis-lymphadenopathy plus syndrome, and has symptoms including hepatomegaly, diabetes insipidus and proptosis. An important gene associated with Histiocytosis is CD207 (CD207 Molecule), and among its related pathways/superpathways are Innate Immune System and Cytokine Signaling in Immune system. The drugs Busulfan and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include bone, thyroid and t cells, and related phenotypes are Increased shRNA abundance (Z-score > 2) and hematopoietic system

Wikipedia : 71 In medicine, histiocytosis refers to an excessive number of histiocytes, (tissue macrophages), and is... more...

Related Diseases for Histiocytosis

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 318)
id Related Disease Score Top Affiliating Genes
1 juvenile xanthogranuloma 32.3 CD163 S100B SERPINA3
2 histiocytosis-lymphadenopathy plus syndrome 12.3
3 benign cephalic histiocytosis 12.2
4 malignant histiocytosis 12.2
5 generalized eruptive histiocytosis 12.2
6 indeterminate cell histiocytosis 12.1
7 histiocytosis, progressive mucinous 12.1
8 progressive nodular histiocytosis 12.1
9 letterer-siwe disease 12.1
10 adult pulmonary langerhans cell histiocytosis 12.0
11 non-langerhans-cell histiocytosis 12.0
12 alk+ histiocytosis 11.9
13 erdheim-chester disease 11.8
14 sea-blue histiocyte disease 11.8
15 eosinophilic granuloma 11.6
16 rosai-dorfman disease 11.6
17 gaucher's disease 11.3
18 niemann-pick disease 11.2
19 langerhans cell sarcoma 11.2
20 hashimoto-pritzker syndrome 11.2
21 papular xanthoma 11.0
22 interdigitating dendritic cell sarcoma 11.0
23 dendritic cell tumor 11.0
24 hemophagocytic lymphohistiocytosis 11.0
25 reticulohistiocytic granuloma 10.9
26 omenn syndrome 10.7
27 polymorphic reticulosis 10.7
28 primary cutaneous anaplastic large cell lymphoma 10.7
29 lymphoma 10.3
30 xanthoma disseminatum 10.3
31 alopecia universalis onychodystrophy vitiligo 10.2 CD1A CD207 FSCN1
32 central nervous system germ cell tumor 10.2 CD163 CD207
33 cavernous sinus meningioma 10.1 CD163 FSCN1 S100B
34 leukemia 10.1
35 malignant giant cell tumor of the tendon sheath 10.1 PTPRC SERPINA3
36 lumbar spinal canal and spinal cord meningioma 10.1 CD207 SMPD1 TNFRSF8
37 adenosquamous colon carcinoma 10.1 CD163 CD207 PTPRC
38 developmental coordination disorder 10.1 CSF1R PTPRC
39 sabinas brittle hair syndrome 10.1 CD163 FSCN1 S100B SLC29A3
40 chiasmal syndrome 10.1 PTPRC S100B
41 benign schwannoma 10.1 PDGFB S100B
42 thyroiditis 10.1
43 diabetes insipidus 10.1
44 sensory organ benign neoplasm 10.0 CD1A PTPRC TNFRSF8
45 dyslexia 10.0 PDGFB S100B SERPINA3
46 rectum lymphoma 10.0 CD1A PTPRC TNFRSF8
47 acromegaly 10.0 CD163 PDGFB SERPINA3
48 tinea favosa 10.0 CD163 CD207 IL1RN SMPD1
49 pulmonary immaturity 10.0 CD163 PTPRC SERPINA3
50 mite infestation 10.0 CD163 PTPRC

Graphical network of the top 20 diseases related to Histiocytosis:



Diseases related to Histiocytosis

Symptoms & Phenotypes for Histiocytosis

Human phenotypes related to Histiocytosis:

56 32 (show all 7)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hepatomegaly 56 32 Occasional (29-5%) HP:0002240
2 diabetes insipidus 56 32 Very frequent (99-80%) HP:0000873
3 proptosis 56 32 Very frequent (99-80%) HP:0000520
4 osteolysis 56 32 Very frequent (99-80%) HP:0002797
5 papule 56 32 Occasional (29-5%) HP:0200034
6 lymphadenopathy 56 32 Occasional (29-5%) HP:0002716
7 pulmonary infiltrates 56 32 Occasional (29-5%) HP:0002113

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

26
id Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-105 9.95 PTPRC SLC29A3
2 Increased shRNA abundance (Z-score > 2) GR00366-A-108 9.95 SLC29A3
3 Increased shRNA abundance (Z-score > 2) GR00366-A-110 9.95 BRAF
4 Increased shRNA abundance (Z-score > 2) GR00366-A-121 9.95 PTPRC
5 Increased shRNA abundance (Z-score > 2) GR00366-A-124 9.95 CD163
6 Increased shRNA abundance (Z-score > 2) GR00366-A-13 9.95 SLC29A3
7 Increased shRNA abundance (Z-score > 2) GR00366-A-137 9.95 SLC29A3 CD163
8 Increased shRNA abundance (Z-score > 2) GR00366-A-149 9.95 CD163 CSF1 PTPRC BRAF SLC29A3
9 Increased shRNA abundance (Z-score > 2) GR00366-A-166 9.95 BRAF
10 Increased shRNA abundance (Z-score > 2) GR00366-A-168 9.95 SLC29A3
11 Increased shRNA abundance (Z-score > 2) GR00366-A-170 9.95 CSF1
12 Increased shRNA abundance (Z-score > 2) GR00366-A-177 9.95 CD163 BRAF
13 Increased shRNA abundance (Z-score > 2) GR00366-A-178 9.95 SLC29A3
14 Increased shRNA abundance (Z-score > 2) GR00366-A-194 9.95 BRAF
15 Increased shRNA abundance (Z-score > 2) GR00366-A-196 9.95 CSF1
16 Increased shRNA abundance (Z-score > 2) GR00366-A-2 9.95 PTPRC
17 Increased shRNA abundance (Z-score > 2) GR00366-A-21 9.95 SLC29A3
18 Increased shRNA abundance (Z-score > 2) GR00366-A-214 9.95 PTPRC
19 Increased shRNA abundance (Z-score > 2) GR00366-A-29 9.95 BRAF
20 Increased shRNA abundance (Z-score > 2) GR00366-A-31 9.95 BRAF
21 Increased shRNA abundance (Z-score > 2) GR00366-A-32 9.95 BRAF
22 Increased shRNA abundance (Z-score > 2) GR00366-A-41 9.95 PTPRC
23 Increased shRNA abundance (Z-score > 2) GR00366-A-47 9.95 CSF1 BRAF
24 Increased shRNA abundance (Z-score > 2) GR00366-A-49 9.95 SLC29A3
25 Increased shRNA abundance (Z-score > 2) GR00366-A-50 9.95 PTPRC
26 Increased shRNA abundance (Z-score > 2) GR00366-A-52 9.95 CD163
27 Increased shRNA abundance (Z-score > 2) GR00366-A-70 9.95 SLC29A3
28 Increased shRNA abundance (Z-score > 2) GR00366-A-74 9.95 CD163
29 Increased shRNA abundance (Z-score > 2) GR00366-A-8 9.95 SLC29A3
30 Increased shRNA abundance (Z-score > 2) GR00366-A-81 9.95 CD163 SLC29A3
31 Increased shRNA abundance (Z-score > 2) GR00366-A-83 9.95 CD163
32 Increased shRNA abundance (Z-score > 2) GR00366-A-9 9.95 CD163
33 Increased shRNA abundance (Z-score > 2) GR00366-A-90 9.95 CSF1

MGI Mouse Phenotypes related to Histiocytosis:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.13 CD101 CD163 CD207 CSF1 CSF1R CSF2
2 cellular MP:0005384 10.11 BRAF CD163 CSF1 CSF1R CSF2 FSCN1
3 homeostasis/metabolism MP:0005376 10 BRAF CD163 CSF1 CSF1R CSF2 IL1RN
4 immune system MP:0005387 10 BRAF CD101 CD163 CD207 CSF1 CSF1R
5 integument MP:0010771 9.56 PDGFB S100B BRAF CSF1 CSF1R CSF2
6 reproductive system MP:0005389 9.23 BRAF CSF1 CSF1R CSF2 IL1RN MAP2K1

Drugs & Therapeutics for Histiocytosis

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

(show top 50) (show all 154)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Busulfan Approved, Investigational Phase 2, Phase 3, Phase 1 55-98-1 2478
2
Cyclophosphamide Approved, Investigational Phase 2, Phase 3, Phase 1 50-18-0, 6055-19-2 2907
3
Cyclosporine Approved, Investigational, Vet_approved Phase 3,Phase 2,Phase 1 79217-60-0, 59865-13-3 5284373 6435893
4
Dexamethasone Approved, Investigational, Vet_approved Phase 3,Phase 2 50-02-2 5743
5
Etoposide Approved Phase 3,Phase 2 33419-42-0 36462
6
Hydrocortisone Approved, Vet_approved Phase 3,Phase 2 50-23-7 5754 657311
7
Methotrexate Approved Phase 3,Phase 2 1959-05-2, 59-05-2 126941
8
Miconazole Approved, Investigational, Vet_approved Phase 3,Phase 2,Phase 1 22916-47-8 4189
9
Mycophenolate mofetil Approved, Investigational Phase 3,Phase 2,Phase 1 128794-94-5 5281078
10
Mycophenolic acid Approved Phase 3,Phase 2,Phase 1 24280-93-1 446541
11
Prednisone Approved, Vet_approved Phase 3,Phase 2 53-03-2 5865
12
Methylprednisolone Approved, Vet_approved Phase 2, Phase 3, Phase 1 83-43-2 6741
13
Prednisolone Approved, Vet_approved Phase 2, Phase 3, Phase 1 50-24-8 5755
14
Vincristine Approved, Investigational Phase 2, Phase 3 2068-78-2, 57-22-7 5978
15
Doxorubicin Approved, Investigational Phase 3,Phase 2 23214-92-8 31703
16
Vinblastine Approved Phase 3,Phase 2 865-21-4 13342 241903
17
Miglustat Approved Phase 3,Phase 2,Phase 1 72599-27-0 51634
18
Cladribine Approved, Investigational Phase 2, Phase 3 4291-63-8 20279
19
Cytarabine Approved, Investigational Phase 2, Phase 3 147-94-4 6253
20
Indomethacin Approved, Investigational Phase 2, Phase 3 53-86-1 3715
21
Asparaginase Approved Phase 3 9015-68-3
22
Pegaspargase Approved, Investigational Phase 3 130167-69-0
23
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3,Phase 2 59-30-3 6037
24
leucovorin Approved, Nutraceutical Phase 3,Phase 2 58-05-9 54575, 6560146 143
25
Butyric Acid Experimental Phase 3,Phase 2 107-92-6 264
26
Doxil Approved June 1999 Phase 3,Phase 2 31703
27
1-Deoxynojirimycin Experimental Phase 3,Phase 2 19130-96-2 1374
28 Alkylating Agents Phase 2, Phase 3, Phase 1
29 Antineoplastic Agents, Alkylating Phase 2, Phase 3, Phase 1
30 Immunosuppressive Agents Phase 2, Phase 3, Phase 1
31 Anti-Bacterial Agents Phase 3,Phase 2,Phase 1
32 Antibiotics, Antitubercular Phase 3,Phase 2,Phase 1
33 Antiemetics Phase 3,Phase 2,Phase 1
34 Antifungal Agents Phase 3,Phase 2,Phase 1
35 Anti-Infective Agents Phase 3,Phase 2,Phase 1
36 Anti-Inflammatory Agents Phase 3,Phase 2,Phase 1
37 Antilymphocyte Serum Phase 3,Phase 2,Phase 1
38 Antimetabolites Phase 3,Phase 2,Phase 1
39 Antimetabolites, Antineoplastic Phase 3,Phase 2,Phase 1
40 Antineoplastic Agents, Hormonal Phase 3,Phase 2,Phase 1
41 Antineoplastic Agents, Phytogenic Phase 3,Phase 2
42 Antirheumatic Agents Phase 3,Phase 2,Phase 1
43 Autonomic Agents Phase 3,Phase 2,Phase 1
44 BB 1101 Phase 3,Phase 2
45 Calcineurin Inhibitors Phase 3,Phase 2,Phase 1
46 Cortisol succinate Phase 3,Phase 2
47 Dermatologic Agents Phase 3,Phase 2,Phase 1
48 Dexamethasone 21-phosphate Phase 3,Phase 2
49 Dexamethasone acetate Phase 3,Phase 2 1177-87-3
50 Etoposide phosphate Phase 3,Phase 2

Interventional clinical trials:

(show top 50) (show all 107)
id Name Status NCT ID Phase
1 T-Cell Depletion and Stem Cell Transplant for Immune Deficiencies and Histiocytic Disorders Unknown status NCT00176826 Phase 2, Phase 3
2 Combination Chemotherapy Followed By Donor Stem Cell Transplant in Treating Patients With Hemophagocytic Lymphohistiocytosis Unknown status NCT00334672 Phase 3
3 H-9926-LCH III: Treatment Protocol of the Third International Study for Langerhans Cell Histiocytosis Completed NCT00488605 Phase 3
4 Application of Miglustat in Patients With Niemann-Pick Type C Completed NCT01760564 Phase 3
5 Stem Cell Transplant for Inborn Errors of Metabolism Completed NCT00176904 Phase 2, Phase 3
6 LCH-IV, International Collaborative Treatment Protocol for Children and Adolescents With Langerhans Cell Histiocytosis Recruiting NCT02205762 Phase 2, Phase 3
7 Vinblastine/Prednisone Versus Single Therapy With Cytarabine for Langerhans Cell Histiocytosis (LCH) Recruiting NCT02670707 Phase 3
8 L-DEP as an Initial Treatment for EBV-HLH Recruiting NCT02912702 Phase 3
9 Splenectomy as a Treatment for Patient With Relapsed Haemophagocytic Lymphohistiocytosis of Unknown Etiology Recruiting NCT02862054 Phase 2, Phase 3
10 L-DEP Regimen as a Salvage Therapy for Refractory Epstein Barr Virus-induced Hemophagocytic Lymphohistiocytosis Recruiting NCT02631109 Phase 3
11 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
12 Study of VTS-270 (2-hydroxypropyl-β-cyclodextrin) to Treat Niemann-Pick Type C1 (NPC1) Disease Recruiting NCT02534844 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
14 Treatment Protocol for Hemophagocytic Lymphohistiocytosis 2004 Active, not recruiting NCT00426101 Phase 3
15 Arimoclomol Prospective Study in Patients Diagnosed With NiemannPick Disease Type C Active, not recruiting NCT02612129 Phase 2, Phase 3
16 A Study to Test the Ability of and Safety of GSK2110183 in Treating Langerhans Cell Histiocytosis Unknown status NCT01395004 Phase 2
17 Umbilical Cord Blood and Placental Blood Transplantation in Treating Patients With Hematologic Cancer or Aplastic Anemia Unknown status NCT00008164 Phase 2
18 Umbilical Cord Blood for Stem Cell Transplantation in Treating Young Patients With Malignant or Nonmalignant Diseases Unknown status NCT00084695 Phase 2
19 Trial of DA-EPOCH Regimen for NHL With HLH Unknown status NCT01818908 Phase 2
20 Treatment of Resistant Langerhans Cell Histiocytosis With ENBREL Completed NCT00048373 Phase 2
21 Study of Sequential Administration of Oral 6-Thioguanine After Methotrexate in Patients With LCH Completed NCT00588536 Phase 2
22 HLA-Nonidentical Stem Cell and Natural Killer Cell Transplantation for Children Less the Two Years of Age With Hematologic Malignancies Completed NCT00145626 Phase 2
23 Stem Cell Transplant for Immunologic or Histiocytic Disorders Completed NCT00176865 Phase 2
24 Hybrid Immunotherapy for Hemophagocytic LymphoHistiocytosis Completed NCT01104025 Phase 2
25 Phase 1/2 Study of Vorinostat Therapy in Niemann-Pick Disease, Type C1 Completed NCT02124083 Phase 1, Phase 2
26 Biomarker Validation for Niemann-Pick Disease, Type C: Safety and Efficacy of N-Acetyl Cysteine Completed NCT00975689 Phase 1, Phase 2
27 Unrelated Hematopoietic Stem Cell Transplantation(HSCT) for Genetic Diseases of Blood Cells Completed NCT00730314 Phase 1, Phase 2
28 Miglustat in Niemann-Pick Type C Disease Completed NCT00517153 Phase 2
29 Phase II Study of Clofarabine in Patients With Recurrent or Refractory Langerhans Cell Histiocytosis Recruiting NCT02425904 Phase 2
30 Methotrexate and Cytosine in Adult Langerhans Cell Histiocytosis Recruiting NCT02389400 Phase 2
31 A Study of Lenalidomide for Adult Histiocyte Disorders Recruiting NCT02523040 Phase 2
32 Single-agent Cobimetinib for Adults With Histiocytic Disorders Recruiting NCT02649972 Phase 2
33 CD34+Selection for Partially Matched Family or Matched Unrelated Adult Donor Transplant Recruiting NCT01049854 Phase 2
34 Treatment of Familiar Lymphohistiocytosis Recruiting NCT02472054 Phase 1, Phase 2
35 Alemtuzumab in Combination With Etoposide and Dexamethasone for the Treatment of Adult Patients With Hemophagocytic Lymphohistiocytosis Recruiting NCT02385110 Phase 2
36 Tocilizumab and Hemophagocytic Lymphohistiocytosis (HLH) Recruiting NCT02007239 Phase 2
37 CD34+ (Malignant) Stem Cell Selection for Patients Receiving Allogenic Stem Cell Transplant Recruiting NCT02061800 Phase 1, Phase 2
38 Study of Pharmacokinetics and Preliminary Efficacy in Patients With Niemann-Pick C1 Recruiting NCT02912793 Phase 1, Phase 2
39 Safety, Tolerability, PK, and Efficacy Evaluation of Repeat Ascending Doses of Olipudase Alfa in Pediatric Patients <18 Years of Age With Acid Sphingomyelinase Deficiency Recruiting NCT02292654 Phase 1, Phase 2
40 Immune Disorder HSCT Protocol Recruiting NCT01821781 Phase 2
41 T Cell Depletion for Recipients of HLA Haploidentical Related Donor Stem Cell Grafts Recruiting NCT00368355 Phase 2
42 Evaluation of Efficacy and Tolerance of Cladribine in Symptomatic Pulmonary Langerhans Cell Histiocytosis Active, not recruiting NCT01473797 Phase 2
43 Reduced Intensity Conditioning for Hemophagocytic Syndromes or Selected Primary Immune Deficiencies (BMT CTN 1204) Active, not recruiting NCT01998633 Phase 2
44 Phase I/II Pilot Study of Mixed Chimerism to Treat Inherited Metabolic Disorders Active, not recruiting NCT01372228 Phase 1, Phase 2
45 A Long-Term Study of Olipudase Alfa in Patients With Acid Sphingomyelinase Deficiency Enrolling by invitation NCT02004704 Phase 2
46 Pediatric MATCH: Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients With Advanced Refractory Solid Tumors or Lymphomas Not yet recruiting NCT03155620 Phase 2
47 Dabrafenib and Trametinib in People With BRAF V600E Mutation Positive Lesions in Erdheim Chester Disease Suspended NCT02281760 Phase 2
48 Reduced Intensity Hematopoietic Cell Transplantation for Patients With Resistant Langerhans Cell Histiocytosis Terminated NCT00618540 Phase 2
49 Blood Stem Cell Transplant in Treating Patients With Hematologic Cancer Terminated NCT00008216 Phase 2
50 Unrelated Donor Transplant for Malignant and Non-Malignant Disorders Terminated NCT01050439 Phase 2

Search NIH Clinical Center for Histiocytosis

Inferred drug relations via UMLS 69 / NDF-RT 48 :


Cochrane evidence based reviews: histiocytosis

Genetic Tests for Histiocytosis

Anatomical Context for Histiocytosis

MalaCards organs/tissues related to Histiocytosis:

39
Bone, Thyroid, T Cells, Bone Marrow, Lung, Skin, B Cells

Publications for Histiocytosis

Articles related to Histiocytosis:

(show top 50) (show all 1524)
id Title Authors Year
1
Physiologic Determinants of Exercise Capacity in Pulmonary Langerhans Cell Histiocytosis: A Multidimensional Analysis. ( 28072848 )
2017
2
Differentiating Intralymphatic Histiocytosis, Intravascular Histiocytosis, and Subtypes of Reactive Angioendotheliomatosis: Review of Clinical and Histologic Features of All Cases Reported to Date. ( 28027079 )
2017
3
Effective BRAF inhibitor vemurafenib therapy in a 2-year-old patient with sequentially diagnosed Langerhans cell histiocytosis and Erdheim-Chester disease. ( 28182116 )
2017
4
Usual and Unusual Manifestations of Familial Hemophagocytic Lymphohistiocytosis and Langerhans Cell Histiocytosis. ( 27894453 )
2017
5
Hematopoietic origin of Langerhans cell histiocytosis and Erdheim Chester disease in adults. ( 28512190 )
2017
6
Langerhans Cell Histiocytosis Presenting as Chronic Otitis Externa. ( 28045847 )
2017
7
Targeting BRAK, MEK, mTOR, and now AKT in histiocytosis: The ongoing revolution of therapies in orphan diseases. ( 28066975 )
2017
8
'Bizarre' rash: adult-onset cutaneous Langerhans cell histiocytosis. ( 28077484 )
2017
9
Dermoscopy of Langerhans cell histiocytosis. ( 28087041 )
2017
10
Langerhans cell histiocytosis associated with lymphoma: an incidental finding that is not associated with BRAF or MAP2K1 mutations. ( 28084334 )
2017
11
BRAF V600 mutations in Langerhans cell histiocytosis with a simple and unique assay. ( 27094161 )
2016
12
Central diabetes insipidus as a very late relapse limited to the pituitary stalk in Langerhans cell histiocytosis. ( 27089406 )
2016
13
Congenital cutaneous Langerhans cell histiocytosis and cutaneous mastocytoma in a child. ( 27617456 )
2016
14
A Fatal Case of Congenital Langerhans Cell Histiocytosis with Disseminated Cutaneous Lesions in a Premature Neonate. ( 27833773 )
2016
15
Langerhans cell histiocytosis with atypical and early neonatal debut. ( 27079402 )
2016
16
Indeterminate cell histiocytosis successfully treated with phototherapy. ( 27547741 )
2016
17
Langerhans cell histiocytosis with nail changes and multisystem disease: a case report. ( 27617727 )
2016
18
Is Langerhans cell histiocytosis a neoplasia? ( 27663873 )
2016
19
Histopathological and Clinical Findings in Cutaneous Manifestation of Erdheim-Chester Disease and Langerhans Cell Histiocytosis Overlap Syndrome Associated With the BRAFV600E Mutation. ( 27898473 )
2016
20
Diverse cutaneous manifestations of Erdheim-Chester disease in a woman with a history of Langerhans cell histiocytosis. ( 27051852 )
2016
21
BRAFV600E and MAP2K1 mutations in Langerhans cell histiocytosis occur predominantly in children. ( 27597420 )
2016
22
A rare case of solitary brain Langerhans cell histiocytosis with intratumoral hemorrhage in a patient affected by Turner syndrome. ( 27127696 )
2016
23
Spontaneous extradural hemorrhage due to Langerhans cell histiocytosis of the skull in a child: A rare presentation. ( 27195034 )
2016
24
Langerhans Cell Histiocytosis and Langerhans Cell Sarcoma: Current Understanding and Differential Diagnosis. ( 27980300 )
2016
25
Localized Langerhans cell histiocytosis masquerading as Brodie's abscess in a 2-year-old child: a case report. ( 27065773 )
2016
26
Successful treatment with cladribine of Erdheim-Chester disease with orbital and central nervous system involvement developing after treatment of Langerhans cell histiocytosis. ( 26964390 )
2016
27
Unilesional, single-system cutaneous Langerhans cell histiocytosis in an 11-month-old infant. ( 27607040 )
2016
28
Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and cirrhosis of the liver: A case report and literature review. ( 27138663 )
2016
29
Late-Onset Langerhans Cell Histiocytosis with Cerebellar Ataxia as an Initial Symptom. ( 27920713 )
2016
30
Clinicopathological features and BRAF(V600E) mutations in patients with isolated hypothalamic-pituitary Langerhans cell histiocytosis. ( 27760550 )
2016
31
Langerhans Cell Histiocytosis of the Maxillary Sinus. ( 27121594 )
2016
32
Langerhans' cell histiocytosis of the temporal fossa: A case report. ( 27073529 )
2016
33
Langerhans cell histiocytosis in the adult lumbar spine: case report. ( 27610317 )
2016
34
BRAF V600E mutation correlates with suppressive tumor immune microenvironment and reduced disease-free survival in Langerhans cell histiocytosis. ( 27622040 )
2016
35
Sinus histiocytosis with massive lymphadenopathy: extra-nodal Rosai-Dorfman disease presenting as a rare aetiology of a large intracardiac mass. ( 27742810 )
2016
36
Pulmonary Langerhans Cell Histiocytosis and Diabetes Insipidus in a Young Smoker. ( 27445532 )
2016
37
(18)F-fluorodeoxyglucose positron emission tomography-computed tomography in the management of adult multisystem Langerhans cell histiocytosis. ( 27645693 )
2016
38
A phase IIa study of afuresertib, an oral pan-AKT inhibitor, in patients with Langerhans cell histiocytosis. ( 27804235 )
2016
39
A Case of Adult Generalized Cutaneous Langerhans Cell Histiocytosis. ( 27081283 )
2016
40
Tertiary lymphoid structures are confined to patients presenting with unifocal Langerhans Cell Histiocytosis. ( 27622056 )
2016
41
Extra Nodal Rosai-Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy) Presenting as Asymmetric Bilateral Optic Atrophy : An Atypical Ocular Presentation. ( 27091209 )
2016
42
Laryngeal Langerhans Cell Histiocytosis Presenting with Neck Mass in an Adult Woman. ( 27127670 )
2016
43
Laryngeal involvement with fatal outcome in progressive nodular histiocytosis: A rare case report. ( 27990389 )
2016
44
Recurrence Pattern of Pulmonary Langerhans Cell Histiocytosis After Lung Transplantation: A Case Report. ( 27932189 )
2016
45
Intralymphatic histiocytosis associated with an orthopedic metal implant. ( 27163920 )
2016
46
Excellent remission rates with limited toxicity in relapsed/refractory Langerhans cell histiocytosis with pulse dexamethasone and lenalidomide in children. ( 27555565 )
2016
47
A Rare Cause of Proptosis in Childhood: Langerhans Cell Histiocytosis. ( 28058158 )
2016
48
Thyroid Langerhans cell histiocytosis and papillary thyroid carcinoma. ( 27867869 )
2016
49
Topical Imiquimod for the Treatment of Childhood Cutaneous Langerhans Cell Histiocytosis. ( 27040152 )
2016
50
A Case Indistinguishable Between Indeterminate Cell Histiocytosis and Cutaneous Rosai-Dorfman Disease. ( 27507795 )
2016

Variations for Histiocytosis

Expression for Histiocytosis

Search GEO for disease gene expression data for Histiocytosis.

Pathways for Histiocytosis

GO Terms for Histiocytosis

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

id Name GO ID Score Top Affiliating Genes
1 extracellular exosome GO:0070062 9.91 CD101 CSF1 FSCN1 IL1RN MAP2K1 PTPRC
2 plasma membrane GO:0005886 9.73 BRAF CD101 CD163 CD1A CD207 CSF1
3 extracellular space GO:0005615 9.7 CSF1 CSF2 IL1RN PDGFB S100B SERPINA3
4 CSF1-CSF1R complex GO:1990682 8.62 CSF1 CSF1R

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

(show all 14)
id Name GO ID Score Top Affiliating Genes
1 positive regulation of cell proliferation GO:0008284 9.83 CSF1 CSF1R CSF2 PDGFB S100B
2 MAPK cascade GO:0000165 9.78 BRAF CSF2 MAP2K1 PDGFB
3 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.62 BRAF CSF1R MAP2K1 PDGFB
4 hemopoiesis GO:0030097 9.58 CSF1 CSF1R PDGFB
5 peptidyl-tyrosine phosphorylation GO:0018108 9.56 CSF1R CSF2 MAP2K1 PDGFB
6 positive regulation of gene expression GO:0010628 9.55 BRAF CSF1 CSF2 MAP2K1 PDGFB
7 face development GO:0060324 9.54 BRAF MAP2K1
8 macrophage differentiation GO:0030225 9.52 CSF1 CSF1R
9 positive regulation of macrophage derived foam cell differentiation GO:0010744 9.51 CSF1 CSF2
10 monocyte differentiation GO:0030224 9.49 CSF1R CSF2
11 positive regulation of podosome assembly GO:0071803 9.46 CSF2 FSCN1
12 regulation of axon regeneration GO:0048679 9.37 BRAF MAP2K1
13 inflammatory response GO:0006954 9.1 CD163 CSF1 CSF1R IL1RN SERPINA3 TNFRSF8
14 macrophage colony-stimulating factor signaling pathway GO:0038145 8.96 CSF1 CSF1R

Sources for Histiocytosis

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