LCH
MCID: LNG108
MIFTS: 63

Langerhans Cell Histiocytosis (LCH)

Categories: Immune diseases, Rare diseases

Aliases & Classifications for Langerhans Cell Histiocytosis

MalaCards integrated aliases for Langerhans Cell Histiocytosis:

Name: Langerhans Cell Histiocytosis 57 76 53 25 37 55 15
Histiocytosis X 12 76 53 25 55
Lch 57 53 25
Langerhans Cell Granulomatosis 12 25
Histiocytosis, Langerhans-Cell 44 73
Langerhans-Cell Histiocytosis 12 15
Hashimoto-Pritzger Disease 25 73
Letterer-Siwe Disease 12 73
Letterer-Siwe Disease Involving Lymph Nodes of Inguinal Region and Lower Limb 12
Letterer-Siwe Disease of Lymph Nodes of Inguinal Region Amd/or Lower Limb 12
Letterer-Siwe Disease of Lymph Nodes of Inguinal Region and/or Lower Limb 12
Letterer-Siwe Disease of Lymph Nodes of Inguinal Region and Lower Limb 12
Letterer-Siwe Disease Involving Lymph Nodes of Axilla and Upper Limb 12
Letterer-Siwe Disease Involving Lymph Nodes of Head, Face, and Neck 12
Letterer-Siwe Disease Involving Lymph Nodes of Head, Face and Neck 12
Letterer-Siwe Disease of Lymph Nodes of Axilla and/or Upper Limb 12
Letterer-Siwe Disease of Lymph Nodes of Head, Face and/or Neck 12
Letterer-Siwe Disease Involving Lymph Nodes of Multiple Sites 12
Letterer-Siwe Disease of Lymph Nodes of Axilla and Upper Limb 12
Letterer-Siwe Disease Involving Intra-Abdominal Lymph Nodes 12
Letterer-Siwe Disease of Lymph Nodes of Head, Face and Neck 12
Letterer-Siwe Disease Involving Intrathoracic Lymph Nodes 12
Letterer-Siwe Disease Involving Intrapelvic Lymph Nodes 12
Letterer-Siwe Disease of Lymph Nodes of Multiple Sites 12
Letterer-Siwe Disease of Intra-Abdominal Lymph Nodes 12
Letterer-Siwe Disease of Intrathoracic Lymph Nodes 12
Letterer-Siwe Disease of Intrapelvic Lymph Nodes 12
Letterer-Siwe Disease Involving Spleen 12
Letterer-Siwe Disease of Spleen 12
Langerhan's Cell Histiocytosis 12
Familial Letterer-Siwe Disease 44

Classifications:



External Ids:

OMIM 57 604856
Disease Ontology 12 DOID:2571
ICD9CM 35 202.5
MedGen 42 C0019621
KEGG 37 H01512

Summaries for Langerhans Cell Histiocytosis

NIH Rare Diseases : 53 Langerhans cell histiocytosis (LCH) is a disorder that primarily affects children, but is also found in adults of all ages. People with LCH produce too many Langerhans cells or histiocytes, a form of white blood cell found in healthy people that is supposed to protect the body from infection. In people with LCH, these cells multiply excessively and build up in certain areas of the body, causing tumors called granulomas to form. The symptoms of LCH vary from person to person, depending on the areas of the body affected. LCH may be found in many areas of the body, including but not limited to the skin and nails, mouth, bones, lymph nodes, pituitary gland, and thyroid gland. When it is found in multiple areas of the body, it is known as multisystem disease. The cause of this disease is unknown, although most data suggest that it is characterized by a growth of immature Langerhans cells that appear to have mutations of the BRAF gene in about half the cases. LCH is not caused by a known infection, is not contagious, nor is it believed to be inherited. There remain differing opinions among experts as to whether it is definitively a cancer or not. Treatment for LCH varies and may include surgery, chemotherapy, radiation therapy, and use of certain medications. LHC includes four variants, with different degrees of severity:Hashimoto-Pritzker disease, a congenital self-healing form Letterer-Siwe disease,  a severe, acute and disseminate form  Hand-Schüller-Christian disease,  an intermediate chronic form with multiple lesions characterized by diabetes insipidus, bulging of the eye and localized lesions in the bone Eosinophilic granuloma, a less severe form, characterized by solitary or few, and chronic lesions of bone or other organs.  Because all the variants have many common symptoms it is thought that they may be manifestations of LCH and not separate syndromes.

MalaCards based summary : Langerhans Cell Histiocytosis, also known as histiocytosis x, is related to non-langerhans-cell histiocytosis and eosinophilic granuloma, and has symptoms including fever An important gene associated with Langerhans Cell Histiocytosis is CD207 (CD207 Molecule), and among its related pathways/superpathways are MAPK signaling pathway and ERK Signaling. The drugs Prednisolone and Prednisolone phosphate have been mentioned in the context of this disorder. Affiliated tissues include lymph node, bone and skin, and related phenotypes are Reduced mammosphere formation and hematopoietic system

Disease Ontology : 12 A histiocytosis that is characterized by clonal proliferation of Langerhans cells.

Genetics Home Reference : 25 Langerhans cell histiocytosis is a disorder in which excess immune system cells called Langerhans cells build up in the body. Langerhans cells, which help regulate the immune system, are normally found throughout the body, especially in the skin, lymph nodes, spleen, lungs, liver, and bone marrow. In Langerhans cell histiocytosis, excess immature Langerhans cells usually form tumors called granulomas. Many researchers now consider Langerhans cell histiocytosis to be a form of cancer, but this classification remains controversial.

OMIM : 57 Langerhans cell histiocytosis (LCH) is a rare disorder characterized by the dysregulated proliferation of Langerhans cells and subsequent organ infiltration. Clinical manifestations range from a spontaneously healing isolated osteolytic lesion to a lymphoma-like syndrome with fatal multiorgan failure, in the absence of any cellular evidence of malignancy. Although the disease can present at any age, the peak age at diagnosis is between 1 and 3 years (summary by Arico et al., 1999). Egeler and D'Angio (1995) presented a classification of histiocytosis syndromes in children: class I, Langerhans cell histiocytosis (LCH); class II, histiocytosis of mononuclear macrophages other than Langerhans cells, including familial hemophagocytic lymphohistiocytosis (267700); and class III, malignant histiocytic disorders, including histiocytic lymphoma. (604856)

Wikipedia : 76 Langerhans cell histiocytosis (LCH) is a rare disease involving clonal proliferation of Langerhans... more...

Related Diseases for Langerhans Cell Histiocytosis

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

(show top 50) (show all 346)
# Related Disease Score Top Affiliating Genes
1 non-langerhans-cell histiocytosis 34.1 BRAF CD163 CD207 F13A1
2 eosinophilic granuloma 33.8 BRAF CD1A
3 histiocytosis 32.7 BRAF CD101 CD163 CD1A CD207 CSF2
4 juvenile xanthogranuloma 32.5 BRAF F13A1 S100B
5 langerhans cell sarcoma 32.5 CD163 CD1A CD207
6 erdheim-chester disease 32.4 BRAF NRAS TNFSF11
7 dendritic cell tumor 32.3 CD1A CD207 IFNG S100B
8 rosai-dorfman disease 30.6 CD163 MAP2K1 S100B
9 nervous system disease 30.3 CSF2 IFNG S100B
10 generalized eruptive histiocytosis 30.0 F13A1 S100B
11 periodontitis 29.9 IFNG IL17A TNFSF11
12 fibroblastic rheumatism 29.9 CD163 F13A1
13 monocytic leukemia 29.8 CSF1 CSF2 IFNG
14 bone inflammation disease 29.7 IFNG IL17A TNFSF11
15 juvenile myelomonocytic leukemia 29.6 BRAF CSF2 NRAS
16 fibrous histiocytoma 29.6 CD163 F13A1 S100B
17 myeloma, multiple 29.5 BRAF CSF2 NRAS TNFSF11
18 aplastic anemia 29.2 CSF1 CSF2 IFNG NRAS
19 adult pulmonary langerhans cell histiocytosis 12.7
20 hashimoto-pritzker syndrome 12.4
21 letterer-siwe disease 12.3
22 leydig cell hypoplasia 12.0
23 lissencephaly with cerebellar hypoplasia 11.9
24 interstitial lung disease 11.6
25 dentin dysplasia, type i 11.3
26 histiocytosis, progressive mucinous 11.3
27 infantile myofibromatosis 11.3
28 reticulohistiocytic granuloma 11.3
29 lissencephaly 2 11.1
30 leydig cell hypoplasia, type i 11.0
31 diabetes insipidus 10.7
32 lipogranulomatosis 10.6
33 lymphoma 10.6
34 reticuloendotheliosis 10.6
35 leukemia 10.5
36 pneumothorax 10.5
37 thyroid cancer, nonmedullary, 1 10.3
38 hemophagocytic lymphohistiocytosis 10.3
39 sarcoma 10.3
40 lymphoma, hodgkin, classic 10.3
41 pulmonary hypertension 10.3
42 sclerosing cholangitis 10.3
43 cholangitis 10.3
44 pneumothorax, primary spontaneous 10.3
45 ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus 10.3
46 dermatitis 10.3
47 cellulitis 10.3
48 scabies 10.3
49 sensorineural hearing loss 10.2
50 adenocarcinoma 10.2

Graphical network of the top 20 diseases related to Langerhans Cell Histiocytosis:



Diseases related to Langerhans Cell Histiocytosis

Symptoms & Phenotypes for Langerhans Cell Histiocytosis

Clinical features from OMIM:

604856

UMLS symptoms related to Langerhans Cell Histiocytosis:


fever

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

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Reduced mammosphere formation GR00396-S 9.1 BRAF CD1A CSF1 F13A1 IL17A NRAS

MGI Mouse Phenotypes related to Langerhans Cell Histiocytosis:

46 (show all 15)
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.27 BRAF CD101 CD163 CD207 CSF1 CSF2
2 homeostasis/metabolism MP:0005376 10.26 BRAF CD163 CSF1 CSF2 F13A1 IFNG
3 endocrine/exocrine gland MP:0005379 10.21 BRAF CSF1 CSF2 F13A1 IFNG IL17A
4 cardiovascular system MP:0005385 10.2 BRAF CD163 CSF1 CSF2 F13A1 IFNG
5 immune system MP:0005387 10.2 BRAF CD101 CD163 CD207 CSF1 CSF2
6 craniofacial MP:0005382 10.14 BRAF CSF1 CSF2 IFNG IL17A MAP2K1
7 integument MP:0010771 10.09 BRAF CSF1 CSF2 IFNG LIF MAP2K1
8 mortality/aging MP:0010768 10.07 BRAF CSF1 CSF2 F13A1 IFNG IL17A
9 digestive/alimentary MP:0005381 10 BRAF IFNG IL17A LIF MAP2K1 NRAS
10 liver/biliary system MP:0005370 9.88 BRAF CD207 CSF1 IFNG LIF NRAS
11 reproductive system MP:0005389 9.86 BRAF CSF1 CSF2 F13A1 IFNG LIF
12 respiratory system MP:0005388 9.85 BRAF CD163 CD207 CSF1 CSF2 F13A1
13 neoplasm MP:0002006 9.8 BRAF CSF2 IFNG MAP2K1 NRAS TNFSF11
14 skeleton MP:0005390 9.61 BRAF CSF1 CSF2 IFNG IL17A LIF
15 vision/eye MP:0005391 9.23 BRAF CD163 CSF1 IFNG LIF MAP2K1

Drugs & Therapeutics for Langerhans Cell Histiocytosis

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

(show top 50) (show all 123)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Prednisolone Approved, Vet_approved Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable 50-24-8 5755
2
Prednisolone phosphate Approved, Vet_approved Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable 302-25-0
3
Methylprednisolone Approved, Vet_approved Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable 83-43-2 6741
4
Methylprednisolone hemisuccinate Approved Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable 2921-57-5
5
Cyclophosphamide Approved, Investigational Phase 2, Phase 3,Phase 1,Not Applicable 6055-19-2, 50-18-0 2907
6
Busulfan Approved, Investigational Phase 2, Phase 3,Phase 1,Early Phase 1,Not Applicable 55-98-1 2478
7
Prednisone Approved, Vet_approved Phase 2, Phase 3,Phase 3,Not Applicable 53-03-2 5865
8
leucovorin Approved Phase 2, Phase 3,Phase 3,Not Applicable 58-05-9 6006 143
9
Vinblastine Approved Phase 2, Phase 3,Phase 3,Not Applicable 865-21-4 241903 13342
10
Cytarabine Approved, Investigational Phase 2, Phase 3,Phase 3 147-94-4 6253
11
Methotrexate Approved Phase 2, Phase 3,Phase 3,Not Applicable 59-05-2, 1959-05-2 126941
12
Indomethacin Approved, Investigational Phase 2, Phase 3 53-86-1 3715
13
Cladribine Approved, Investigational Phase 2, Phase 3 4291-63-8 20279
14
Vincristine Approved, Investigational Phase 2, Phase 3 57-22-7, 2068-78-2 5978
15
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2, Phase 3,Phase 3,Not Applicable 59-30-3 6037
16
Prednisolone hemisuccinate Experimental Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable 2920-86-7
17 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
18 Hormone Antagonists Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
19 Immunologic Factors Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
20 Antimetabolites, Antineoplastic Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
21 Hormones Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
22 Prednisolone acetate Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
23 Antirheumatic Agents Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
24 glucocorticoids Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
25 Immunosuppressive Agents Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
26 Antimetabolites Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
27 Antineoplastic Agents, Hormonal Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
28 Anti-Inflammatory Agents Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
29 Methylprednisolone acetate Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
30 Peripheral Nervous System Agents Phase 2, Phase 3,Phase 1,Not Applicable
31 Antineoplastic Agents, Alkylating Phase 2, Phase 3,Phase 1,Not Applicable
32 Alkylating Agents Phase 2, Phase 3,Phase 1,Not Applicable
33 Dermatologic Agents Phase 2, Phase 3,Phase 3,Not Applicable
34 Analgesics, Non-Narcotic Phase 2, Phase 3
35 Anti-Inflammatory Agents, Non-Steroidal Phase 2, Phase 3
36 Antibodies Phase 2, Phase 3
37 Analgesics Phase 2, Phase 3
38 2-chloro-3'-deoxyadenosine Phase 2, Phase 3
39 Vitamin B9 Phase 2, Phase 3,Phase 3,Not Applicable
40 Vitamin B Complex Phase 2, Phase 3,Phase 3,Not Applicable
41 Antiviral Agents Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
42 Immunoglobulins, Intravenous Phase 2, Phase 3
43 gamma-Globulins Phase 2, Phase 3
44 Antimitotic Agents Phase 2, Phase 3,Phase 3,Not Applicable
45 Folic Acid Antagonists Phase 2, Phase 3,Phase 3,Not Applicable
46 6-Mercaptopurine Phase 2, Phase 3,Phase 3
47 Antineoplastic Agents, Phytogenic Phase 2, Phase 3,Phase 3,Not Applicable
48 Nucleic Acid Synthesis Inhibitors Phase 2, Phase 3,Phase 3,Not Applicable
49 Folate Phase 2, Phase 3,Phase 3,Not Applicable
50 Rho(D) Immune Globulin Phase 2, Phase 3

Interventional clinical trials:

(show top 50) (show all 51)
# Name Status NCT ID Phase Drugs
1 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
2 Vinblastine/Prednisone Versus Single Therapy With Cytarabine for Langerhans Cell Histiocytosis (LCH) Recruiting NCT02670707 Phase 3 Cytarabine;Vinblastine/prednisone
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 T-Cell Depletion and Stem Cell Transplant for Immune Deficiencies and Histiocytic Disorders Terminated NCT00176826 Phase 2, Phase 3 Myeloablative conditioning regimen
5 Umbilical Cord Blood and Placental Blood Transplantation in Treating Patients With Hematologic Cancer or Aplastic Anemia Unknown status NCT00008164 Phase 2
6 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
7 Study of Clofarabine in Patients With Recurrent or Refractory Langerhans Cell Histiocytosis and LCH-related Disorders Recruiting NCT02425904 Phase 2 Clofarabine
8 Methotrexate and Cytosine in Adult Langerhans Cell Histiocytosis Completed NCT02389400 Phase 2 methotrexate
9 A Study to Test the Ability of and Safety of GSK2110183 in Treating Langerhans Cell Histiocytosis Completed NCT01395004 Phase 2 GSK2110183
10 Evaluation of Efficacy and Tolerance of Cladribine in Symptomatic Pulmonary Langerhans Cell Histiocytosis Active, not recruiting NCT01473797 Phase 2 Cladribine
11 Reduced Intensity Hematopoietic Cell Transplantation for Patients With Resistant Langerhans Cell Histiocytosis Terminated NCT00618540 Phase 2 fludarabine phosphate;melphalan
12 Treatment of Resistant Langerhans Cell Histiocytosis With ENBREL Completed NCT00048373 Phase 2 Etanercept
13 Denosumab for the Treatment of Adult LCH Recruiting NCT03270020 Phase 2 Denosumab 70 MG/ML [Xgeva]
14 Study of Sequential Administration of Oral 6-Thioguanine After Methotrexate in Patients With LCH Completed NCT00588536 Phase 2 Methotrexate;6-Thioguanine;Leucovorin Calcium
15 Clofarabine for Langerhans in Pedi Withdrawn NCT01796405 Phase 2 Clofarabine
16 A Combination of Vemurafenib, Cytarabine and 2-chlorodeoxyadenosine in Children With LCH and BRAF V600E Mutation Recruiting NCT03585686 Phase 2 Vemurafenib;Cytarabine;2-chlorodeoxyadenosine
17 A Study of Lenalidomide for Adult Histiocyte Disorders Active, not recruiting NCT02523040 Phase 2 Lenalidomide
18 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
19 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
20 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
21 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
22 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
23 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
24 Tazemetostat in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With EZH2, SMARCB1, or SMARCA4 Gene Mutations (A Pediatric MATCH Treatment Trial) Suspended NCT03213665 Phase 2 Tazemetostat
25 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
26 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)
27 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
28 Deferasirox in Treating Iron Overload Caused By Blood Transfusions in Patients With Hematologic Malignancies Completed NCT01273766 Phase 2 deferasirox
29 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
30 A Study of Vemurafenib in Participants With BRAF V600 Mutation-Positive Cancers Completed NCT01524978 Phase 2 cetuximab;vemurafenib;vemurafenib
31 A Study to Determine Safety, Tolerability and Pharmacokinetics of Oral Dabrafenib In Children and Adolescent Subjects Recruiting NCT01677741 Phase 1 Dabrafenib
32 Indium In 111 Pentetreotide in Treating Patients With Refractory Cancer Terminated NCT00002947 Phase 1
33 Sunitinib Malate in Treating HIV-Positive Patients With Cancer Receiving Antiretroviral Therapy Completed NCT00890747 Phase 1 sunitinib malate
34 Study to Investigate Safety, Pharmacokinetic (PK), Pharmacodynamic (PD) and Clinical Activity of Trametinib in Subjects With Cancer or Plexiform Neurofibromas and Trametinib in Combination With Dabrafenib in Subjects With Cancers Harboring V600 Mutations Recruiting NCT02124772 Phase 1 Trametinib;Dabrafenib
35 Cord Blood Fucosylation to Enhance Homing and Engraftment in Patients With Hematologic Malignancies Completed NCT01471067 Phase 1 Melphalan;Fludarabine;Mycophenolate mofetil;Tacrolimus;Rituximab;ATG;Busulfan;Clofarabine
36 Cardiovascular Risk Factors and LCH in Adults Unknown status NCT00483925
37 Assessment of Safety of Air Travel in Patients With Pulmonary Langerhans Cell Histiocytosis Recruiting NCT03052101
38 Combination Chemotherapy in Treating Young Patients With Langerhans Cell Histiocytosis Completed NCT00276757 Not Applicable leucovorin calcium;methotrexate;prednisolone;vinblastine sulfate
39 Prospective Evaluation of Adult Pulmonary Langerhans Cell Histiocytosis Completed NCT01225601
40 Evaluation of Outcome of Adult Pulmonary Langerhans Cell Histiocytosis Based on Lung HRCT and Lung Function Completed NCT01651507
41 Characterization of Pulmonary Langerhans Cell Histiocytosis Recruiting NCT03093727
42 Evaluation of Exercise Capacity and Exercise Limitation in Patients With Pulmonary Langerhans Cell Histiocytosis Recruiting NCT02665546
43 Ceftriaxone in Non-neutropenic Fever Completed NCT01225718
44 A Study of Memory, Thinking, and Brain Imaging in Adults With Histiocytosis Recruiting NCT03127709
45 Uptake and Biodistribution of 18F-fluorocholine in Histiocytic Disorders by PET Imaging and Biopsy Measurement Completed NCT02608619
46 Blood Stem Cell Transplant in Treating Patients With Hematologic Cancer Terminated NCT00008216
47 CD34+ (Non-Malignant) Stem Cell Selection for Patients Receiving Allogeneic Stem Cell Transplantation Recruiting NCT01966367 Early Phase 1
48 Project: Every Child for Younger Patients With Cancer Recruiting NCT02402244
49 Collecting and Storing Tissue From Young Patients With Cancer Completed NCT00898755
50 Allogeneic Hematopoietic Stem Cell Transplant for Patients With Primary Immune Deficiencies Recruiting NCT01652092 Not Applicable Alemtuzumab 0.3 mg;Cyclophosphamide;Busulfan;Fludarabine phosphate 40 mg;Melphalan;Alemtuzumab 0.2 mg;Busulfan;Fludarabine phosphate 30 mg;MESNA

Search NIH Clinical Center for Langerhans Cell Histiocytosis

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


Cochrane evidence based reviews: histiocytosis, langerhans-cell

Genetic Tests for Langerhans Cell Histiocytosis

Anatomical Context for Langerhans Cell Histiocytosis

MalaCards organs/tissues related to Langerhans Cell Histiocytosis:

41
Lymph Node, Bone, Skin, Lung, Spleen, Thyroid, Bone Marrow

Publications for Langerhans Cell Histiocytosis

Articles related to Langerhans Cell Histiocytosis:

(show top 50) (show all 2077)
# 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
A novel fusion gene PLEKHA6-NTRK3 in langerhans cell histiocytosis. ( 30098202 )
2019
4
Reconstruction of a severe mandibular pathological fracture caused by Langerhans cell histiocytosis using a free fibula osteocutaneous flap:a case report. ( 29372175 )
2018
5
An update on the treatment of pediatric-onset Langerhans cell histiocytosis through pharmacotherapy. ( 29355432 )
2018
6
The use of BRAF V600E mutation-specific immunohistochemistry in pediatric Langerhans cell histiocytosis. ( 28219109 )
2018
7
A Rare Case of Erdheim-Chester Disease (Non-Langerhans Cell Histiocytosis) with Concurrent Langerhans Cell Histiocytosis: A Diagnostic and Therapeutic Challenge. ( 29888013 )
2018
8
Langerhans cell histiocytosis mimicking molluscum contagiosum: A case series. ( 29528195 )
2018
9
Oral manifestation of Langerhans cell histiocytosis: a case report. ( 29884166 )
2018
10
Reflectance Confocal Microscopy for the Diagnosis of Langerhans Cell Histiocytosis. ( 29341062 )
2018
11
A case report of orbital Langerhans cell histiocytosis presenting as a orbital cellulitis. ( 29643005 )
2018
12
Classic Hodgkin lymphoma with osseous involvement mimicking Langerhans cell histiocytosis in a child. ( 29307624 )
2018
13
Rosai-Dorfman Disease: Rare Pulmonary Involvement Mimicking Pulmonary Langerhans Cell Histiocytosis and Review of the Literature. ( 29850355 )
2018
14
Outcome After Radiation Therapy for Langerhans Cell Histiocytosis Is Dependent on Site of Involvement. ( 29413280 )
2018
15
Bone metabolism in Langerhans cell histiocytosis. ( 29967185 )
2018
16
Langerhans cell histiocytosis of the orbit: A study of eight cases. ( 29930447 )
2018
17
National Jewish Health Classical Pulmonary Radiology Case Reports: Imaging Features of Langerhans Cell Histiocytosis. ( 29878226 )
2018
18
Radiotherapy for Langerhans cell histiocytosis with paraplegia: A rare oncologic emergency case report in infancy and literature review. ( 29907475 )
2018
19
Congenital Langerhans cell histiocytosis presenting in a 27-week-gestation neonate. ( 29436047 )
2018
20
MRI features in solitary cerebral Langerhans cell histiocytosis : a case report and review of literature. ( 29929024 )
2018
21
Langerhans cell histiocytosis in an 18-month-old child presenting as periorbital cellulitis. ( 29755272 )
2018
22
Langerhans cell histiocytosis of bone in an adult: A case report. ( 29904462 )
2018
23
Intraoperative MRI-guided biopsy in the diagnosis of suprasellar Langerhans Cell Histiocytosis. ( 29317365 )
2018
24
Immune Thrombocytopenia in a Child With Refractory Langerhans Cell Histiocytosis Following Cladribine Containing Therapy. ( 29889804 )
2018
25
Pulmonary Langerhans cell histiocytosis, acute myeloid leukemia, and myelofibrosis in a large family and review of the literature. ( 29428448 )
2018
26
Pulmonary Langerhans Cell Histiocytosis(PLCH) masquerading as Atypical Mycobacterial infection. ( 29942736 )
2018
27
Langerhans Cell Histiocytosis of the Adult Cervical Spine: A Case Report and Literature Review. ( 29890538 )
2018
28
Localised Langerhans cell histiocytosis of the hypothalamic-pituitary region: case report and literature review. ( 29858859 )
2018
29
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
30
RAF/MEK/extracellular signal-related kinase pathway suppresses dendritic cell migration and traps dendritic cells in Langerhans cell histiocytosis lesions. ( 29263218 )
2018
31
A unique manifestation of Langerhans cell histiocytosis: Diagnostic and therapeutic considerations of atypical cases. ( 29940687 )
2018
32
Isolated Langerhans Cell Histiocytosis of Orbit: A Case Report and Review of the Literature. ( 29850325 )
2018
33
Juvenile Xanthogranuloma in a Pediatric Patient with Langerhans Cell Histiocytosis. ( 29765943 )
2018
34
A case of adult onset folliculocentric Langerhans cell histiocytosis. ( 29924409 )
2018
35
Successful management of multiple-systemic Langerhans cell histiocytosis involving endocrine organs in an adult: A case report and review of literature. ( 29952977 )
2018
36
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
37
A Case of Unicentric Castleman Disease and Langerhans Cell Histiocytosis: Two Entities in One Lymph Node. ( 30549992 )
2018
38
Occult Langerhans Cell Histiocytosis in Clear Cell Renal Cell Carcinoma. ( 29017411 )
2018
39
Langerhans cell histiocytosis presenting as Crohn's disease: a case report. ( 29737419 )
2018
40
Granulomatous dermatitis secondary to vemurafenib in a child with Langerhans cell histiocytosis. ( 30216522 )
2018
41
Systemic Congenital Langerhans Cell Histiocytosis Masquerading as Diffuse Neonatal Hemangiomatosis. ( 30129550 )
2018
42
Dramatic efficacy of dabrafenib in Erdheim-Chester disease (ECD): a pediatric patient with multiple large intracranial ECD lesions hidden by refractory Langerhans cell histiocytosis. ( 30265230 )
2018
43
Extrapontine myelinolysis effects in intracranial langerhans cell histiocytosis: Case report. ( 30211327 )
2018
44
A very rare case of a right insular lobe Langerhans cell histiocytosis (CD1a +) mimicking glioblastoma multiforme in a young adult. ( 30261373 )
2018
45
Langerhans cell histiocytosis and primary hemophagocytic lymphohistiocytosis with persistent clonal T-large granular lymphocyte proliferation. ( 29582110 )
2018
46
Langerhans cell histiocytosis complicated with hemophagocytic lymphohistiocytosis in a boy with a novel XIAP mutation: A case report. ( 30383659 )
2018
47
A multicenter study of patients with multisystem Langerhans cell histiocytosis who develop secondary hemophagocytic lymphohistiocytosis. ( 30521100 )
2018
48
Pituitary gland and bone involvement of Langerhans cell histiocytosis in a boy and brief review of the literature. ( 30006648 )
2018
49
Langerhans Cell Histiocytosis Associated With Underlying Hematolymphoid Disorders in Adults: Report of 2 Cases and Review of the Literature. ( 30035752 )
2018
50
Morphometric Study of Pulmonary Arterial Changes in Pulmonary Langerhans Cell Histiocytosis. ( 30040456 )
2018

Variations for Langerhans Cell Histiocytosis

Expression for Langerhans Cell Histiocytosis

Search GEO for disease gene expression data for Langerhans Cell Histiocytosis.

Pathways for Langerhans Cell Histiocytosis

Pathways related to Langerhans Cell Histiocytosis according to KEGG:

37
# Name Kegg Source Accession
1 MAPK signaling pathway hsa04010

Pathways related to Langerhans Cell Histiocytosis according to GeneCards Suite gene sharing:

(show all 48)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.77 BRAF CSF1 CSF2 IL17A LIF MAP2K1
2
Show member pathways
13.53 BRAF CSF1 CSF2 IFNG IL17A LIF
3
Show member pathways
13.4 BRAF CD101 CD1A CD207 CSF1 CSF2
4
Show member pathways
13.32 CSF1 CSF2 IL17A LIF NRAS TNFSF11
5
Show member pathways
13.19 CSF1 CSF2 IL17A LIF NRAS TNFSF11
6
Show member pathways
12.96 BRAF CSF1 CSF2 F13A1 IFNG IL17A
7
Show member pathways
12.6 BRAF IFNG MAP2K1 NRAS
8
Show member pathways
12.38 BRAF CSF2 IFNG MAP2K1 NRAS
9
Show member pathways
12.33 BRAF MAP2K1 NRAS TNFSF11
10
Show member pathways
12.26 CSF2 IFNG LIF MAP2K1 NRAS
11
Show member pathways
12.21 CSF2 IFNG IL17A MAP2K1 NRAS
12
Show member pathways
12.15 BRAF MAP2K1 NRAS TNFSF11
13
Show member pathways
12.06 BRAF MAP2K1 NRAS
14 12.01 LIF MAP2K1 NRAS
15
Show member pathways
12 BRAF MAP2K1 NRAS
16
Show member pathways
11.99 BRAF MAP2K1 NRAS
17
Show member pathways
11.99 IFNG IL17A MAP2K1 NRAS
18 11.96 CSF1 IFNG MAP2K1 TNFSF11
19 11.91 BRAF MAP2K1 NRAS
20 11.9 F13A1 IL17A LIF
21 11.89 BRAF MAP2K1 NRAS
22 11.88 BRAF MAP2K1 NRAS
23 11.86 BRAF MAP2K1 TNFSF11
24 11.85 BRAF IFNG MAP2K1
25 11.84 CD163 CSF1 CSF2 IFNG
26 11.8 IFNG MAP2K1 NRAS
27 11.8 CD1A CSF1 CSF2
28 11.8 CSF1 CSF2 LIF MAP2K1
29 11.71 CD101 IFNG IL17A
30
Show member pathways
11.71 BRAF MAP2K1 NRAS
31 11.65 BRAF MAP2K1 NRAS
32 11.61 CSF1 CSF2 IFNG IL17A TNFSF11
33
Show member pathways
11.59 BRAF MAP2K1 NRAS
34
Show member pathways
11.55 BRAF IFNG MAP2K1
35 11.54 BRAF MAP2K1 NRAS
36 11.52 CD163 CSF2 IFNG IL17A
37 11.44 CSF1 CSF2 LIF
38 11.37 BRAF MAP2K1 NRAS
39 11.3 BRAF LIF MAP2K1 NRAS
40 11.27 BRAF MAP2K1 NRAS
41 11.16 CSF1 CSF2 IFNG
42 11.05 BRAF MAP2K1
43 10.98 MAP2K1 NRAS
44 10.97 CD163 CD1A CD207 CSF1 CSF2 IFNG
45 10.96 BRAF MAP2K1
46 10.95 CSF1 CSF2 IL17A LIF TNFSF11
47 10.81 CSF1 CSF2 IL17A LIF MAP2K1 TNFSF11
48 10.76 CSF1 CSF2

GO Terms for Langerhans Cell Histiocytosis

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

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.5 CSF1 CSF2 IFNG IL17A LIF S100B
2 extracellular region GO:0005576 9.28 CD163 CSF1 CSF2 F13A1 IFNG IL17A

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

(show all 15)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of cell proliferation GO:0008284 9.89 CSF1 CSF2 IFNG LIF S100B
2 immune response GO:0006955 9.85 CD1A CSF2 IFNG IL17A LIF TNFSF11
3 regulation of signaling receptor activity GO:0010469 9.8 CSF1 CSF2 IFNG IL17A LIF TNFSF11
4 MAPK cascade GO:0000165 9.73 BRAF CSF2 MAP2K1 NRAS
5 cytokine-mediated signaling pathway GO:0019221 9.73 CSF1 CSF2 F13A1 IL17A LIF TNFSF11
6 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.65 CSF2 IFNG LIF
7 positive regulation of peptidyl-serine phosphorylation of STAT protein GO:0033141 9.55 IFNG LIF
8 face development GO:0060324 9.54 BRAF MAP2K1
9 positive regulation of macrophage derived foam cell differentiation GO:0010744 9.52 CSF1 CSF2
10 positive regulation of macrophage differentiation GO:0045651 9.51 CSF1 LIF
11 positive regulation of gene expression GO:0010628 9.5 BRAF CSF1 CSF2 IFNG LIF MAP2K1
12 regulation of axon regeneration GO:0048679 9.46 BRAF MAP2K1
13 osteoclast proliferation GO:0002158 9.4 CSF1 TNFSF11
14 positive regulation of interleukin-23 production GO:0032747 9.33 CSF2 IFNG IL17A
15 positive regulation of osteoclast differentiation GO:0045672 8.92 CSF1 IFNG IL17A TNFSF11

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

# Name GO ID Score Top Affiliating Genes
1 cytokine activity GO:0005125 9.1 CSF1 CSF2 IFNG IL17A LIF TNFSF11

Sources for Langerhans Cell Histiocytosis

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
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