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 58 77 54 26 38 30 56 15 17
Histiocytosis X 12 77 54 26 56
Lch 58 54 26
Langerhans Cell Granulomatosis 12 26
Histiocytosis, Langerhans-Cell 45 74
Langerhans-Cell Histiocytosis 12 15
Hashimoto-Pritzger Disease 26 74
Letterer-Siwe Disease 12 74
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 45

Classifications:



External Ids:

Disease Ontology 12 DOID:2571
OMIM 58 604856
KEGG 38 H01512
ICD9CM 36 202.5
MedGen 43 C0019621

Summaries for Langerhans Cell Histiocytosis

NIH Rare Diseases : 54 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 eosinophilic granuloma and non-langerhans-cell histiocytosis, and has symptoms including fever An important gene associated with Langerhans Cell Histiocytosis is CD1A (CD1a Molecule), and among its related pathways/superpathways are MAPK signaling pathway and PEDF Induced Signaling. The drugs Prednisolone phosphate and Prednisolone 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 : 26 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 : 58 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 : 77 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 376)
# Related Disease Score Top Affiliating Genes
1 eosinophilic granuloma 34.0 BRAF CD1A
2 non-langerhans-cell histiocytosis 33.7 BRAF CD163 CD207 F13A1
3 erdheim-chester disease 32.9 BRAF NRAS
4 dendritic cell tumor 32.5 CD1A CD207 S100B
5 juvenile xanthogranuloma 32.4 BRAF F13A1 S100B
6 langerhans cell sarcoma 32.3 CD163 CD1A CD207
7 histiocytosis 31.8 BRAF CD101 CD163 CD1A CD207 CSF2
8 reticulohistiocytic granuloma 31.7 CD163 CD207
9 rosai-dorfman disease 30.3 CD163 MAP2K1 S100B
10 generalized eruptive histiocytosis 30.0 F13A1 S100B
11 juvenile myelomonocytic leukemia 29.9 BRAF CSF2 NRAS
12 myeloma, multiple 29.8 BRAF CSF2 NRAS TNFSF11
13 pigmented villonodular synovitis 29.7 CSF1 TNFSF11
14 fibroblastic rheumatism 29.7 CD163 F13A1
15 histiocytic sarcoma 29.7 BRAF CD163 CSF1
16 fibrous histiocytoma 29.3 CD163 F13A1 S100B
17 adult pulmonary langerhans cell histiocytosis 12.7
18 hashimoto-pritzker syndrome 12.5
19 letterer-siwe disease 12.3
20 leydig cell hypoplasia 12.0
21 lissencephaly with cerebellar hypoplasia 11.9
22 interstitial lung disease 11.6
23 dentin dysplasia, type i 11.3
24 histiocytosis, progressive mucinous 11.3
25 infantile myofibromatosis 11.3
26 lissencephaly 2 11.1
27 leydig cell hypoplasia, type i 11.1
28 diabetes insipidus 10.8
29 leukemia 10.7
30 lipogranulomatosis 10.6
31 lymphoma 10.6
32 reticuloendotheliosis 10.6
33 pneumothorax 10.6
34 thyroid cancer 10.5
35 sclerosing cholangitis 10.5
36 cholangitis 10.5
37 thyroid cancer, nonmedullary, 1 10.4
38 sarcoma 10.4
39 pulmonary hypertension 10.4
40 hemophagocytic lymphohistiocytosis 10.4
41 lymphoma, hodgkin, classic 10.3
42 leukemia, acute myeloid 10.3
43 ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus 10.3
44 lymphocytic leukemia 10.3
45 dermatitis 10.3
46 adenocarcinoma 10.3
47 cellulitis 10.3
48 myeloid leukemia 10.3
49 pneumothorax, primary spontaneous 10.3
50 leukemia, acute lymphoblastic 10.3

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:

27
# 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:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.13 BRAF CD101 CD163 CD207 CSF1 CSF2
2 homeostasis/metabolism MP:0005376 10.1 BRAF CD163 CSF1 CSF2 F13A1 IL17A
3 endocrine/exocrine gland MP:0005379 10.06 BRAF CSF1 CSF2 F13A1 IL17A MAP2K1
4 craniofacial MP:0005382 10.02 BRAF CSF1 CSF2 IL17A MAP2K1 NRAS
5 immune system MP:0005387 10.02 BRAF CD101 CD163 CD207 CSF1 CSF2
6 integument MP:0010771 9.87 BRAF CSF1 CSF2 MAP2K1 NRAS S100B
7 respiratory system MP:0005388 9.76 BRAF CD163 CD207 CSF1 CSF2 F13A1
8 neoplasm MP:0002006 9.65 BRAF CSF2 MAP2K1 NRAS TNFSF11
9 skeleton MP:0005390 9.5 BRAF CSF1 CSF2 IL17A MAP2K1 NRAS
10 vision/eye MP:0005391 9.1 BRAF CD163 CSF1 MAP2K1 NRAS TNFSF11

Drugs & Therapeutics for Langerhans Cell Histiocytosis

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

(show top 50) (show all 127)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Prednisolone phosphate Approved, Vet_approved Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable 302-25-0
2
Prednisolone Approved, Vet_approved Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable 50-24-8 5755
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
Busulfan Approved, Investigational Phase 2, Phase 3,Phase 1,Early Phase 1,Not Applicable 55-98-1 2478
6
Cyclophosphamide Approved, Investigational Phase 2, Phase 3,Phase 1,Not Applicable 6055-19-2, 50-18-0 2907
7
Mercaptopurine Approved Phase 2, Phase 3,Phase 3 50-44-2 667490
8
Vincristine Approved, Investigational Phase 2, Phase 3 2068-78-2, 57-22-7 5978
9
Methotrexate Approved Phase 2, Phase 3,Phase 3,Not Applicable 1959-05-2, 59-05-2 126941
10
Prednisone Approved, Vet_approved Phase 2, Phase 3,Phase 3,Not Applicable 53-03-2 5865
11
Cytarabine Approved, Experimental, Investigational Phase 2, Phase 3,Phase 3 147-94-4, 65-46-3 6253
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
Vinblastine Approved Phase 2, Phase 3,Phase 3,Not Applicable 865-21-4 13342 241903
15
leucovorin Approved Phase 2, Phase 3,Phase 3,Not Applicable 58-05-9 6006 143
16
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2, Phase 3,Phase 3,Not Applicable 59-30-3 6037
17
Prednisolone hemisuccinate Experimental Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable 2920-86-7
18 Prednisolone acetate Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
19 Peripheral Nervous System Agents Phase 2, Phase 3,Phase 1,Not Applicable
20 Anti-Inflammatory Agents Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
21 Antimetabolites Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
22 Hormones Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
23 Immunosuppressive Agents Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
24 Antimetabolites, Antineoplastic Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
25 Antineoplastic Agents, Hormonal Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
26 Hormone Antagonists Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
27 Immunologic Factors Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
28 Methylprednisolone Acetate Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
29 Antirheumatic Agents Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
30 glucocorticoids Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
31 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
32 Alkylating Agents Phase 2, Phase 3,Phase 1,Not Applicable
33 Antineoplastic Agents, Alkylating Phase 2, Phase 3,Phase 1,Not Applicable
34 Immunoglobulins Phase 2, Phase 3
35 Rho(D) Immune Globulin Phase 2, Phase 3
36 Antiviral Agents Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
37 Tocolytic Agents Phase 2, Phase 3
38 gamma-Globulins Phase 2, Phase 3
39 Cyclooxygenase Inhibitors Phase 2, Phase 3
40 Vitamin B Complex Phase 2, Phase 3,Phase 3,Not Applicable
41 Anti-Infective Agents Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
42 Anti-Inflammatory Agents, Non-Steroidal Phase 2, Phase 3
43 Immunoglobulins, Intravenous Phase 2, Phase 3
44 Antibodies Phase 2, Phase 3
45 Nucleic Acid Synthesis Inhibitors Phase 2, Phase 3,Phase 3,Not Applicable
46 Antimitotic Agents Phase 2, Phase 3,Phase 3,Not Applicable
47 Vitamin B9 Phase 2, Phase 3,Phase 3,Not Applicable
48 Analgesics Phase 2, Phase 3
49 Folate Phase 2, Phase 3,Phase 3,Not Applicable
50 Antineoplastic Agents, Phytogenic Phase 2, Phase 3,Phase 3,Not Applicable

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 Assessment of Safety of Air Travel in Patients With Pulmonary Langerhans Cell Histiocytosis Unknown status NCT03052101
37 Cardiovascular Risk Factors and LCH in Adults Unknown status NCT00483925
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 Completed 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 74 / NDF-RT 52 :


Cochrane evidence based reviews: histiocytosis, langerhans-cell

Genetic Tests for Langerhans Cell Histiocytosis

Genetic tests related to Langerhans Cell Histiocytosis:

# Genetic test Affiliating Genes
1 Langerhans Cell Histiocytosis 30

Anatomical Context for Langerhans Cell Histiocytosis

MalaCards organs/tissues related to Langerhans Cell Histiocytosis:

42
Lymph Node, Bone, Skin, Thyroid, Lung, Spleen, Liver

Publications for Langerhans Cell Histiocytosis

Articles related to Langerhans Cell Histiocytosis:

(show top 50) (show all 3017)
# Title Authors Year
1
Cutaneous adult xanthogranuloma with a small portion of BRAFV600E mutated Langerhans cell histiocytosis populations: A case report and the review of published work. ( 30536719 )
2019
2
Central diabetes insipidus in pediatric patients with Langerhans cell histiocytosis: Results from the JLSG-96/02 studies. ( 30207064 )
2019
3
Langerhans Cell Histiocytosis Presenting as Enterocolitis and Shock in Neonate. ( 30720676 )
2019
4
Cladribine-related immunosuppression may have fostered graft-versus-host disease after lung transplant for pulmonary Langerhans cell histiocytosis. ( 30270491 )
2019
5
Langerhans Cell Histiocytosis With Vertebral Involvement Diagnosed and Treated Over the Last 15 Years in a Single Canadian Pediatric Academic Institution. ( 30855315 )
2019
6
Langerhans cell sarcoma arising from antecedent langerhans cell histiocytosis: A case report. ( 30855438 )
2019
7
Establishment of cell line and in vivo mouse model of canine Langerhans cell histiocytosis. ( 30884050 )
2019
8
Langerhans cell histiocytosis presenting as eosinophilic granuloma of the bilateral forearms in an 8-year-old girl: a case report. ( 30885248 )
2019
9
Bilateral choroidal osteoma associated with langerhans cell histiocytosis, a coincidence? ( 30899858 )
2019
10
The langerhans cell histiocytosis in oral and maxillofacial area. ( 30900679 )
2019
11
A novel fusion gene PLEKHA6-NTRK3 in langerhans cell histiocytosis. ( 30098202 )
2019
12
Langerhans Cell Histiocytosis with Lymph Node Involvement Presenting as Erythroderma. ( 30182134 )
2019
13
Langerhans cell histiocytosis: a rare case of large-bowel inflammatory lesions. ( 30469151 )
2019
14
Langerhans Cell Histiocytosis of the Skull in a Burr Hole Site Covered with Hydroxyapatite Material. ( 30503292 )
2019
15
Langerhans cell histiocytosis in adults is associated with a high prevalence of hematologic and solid malignancies. ( 30597769 )
2019
16
Langerhans cell histiocytosis presenting as a rapidly evolving frontotemporal syndrome. ( 30631989 )
2019
17
Isolated hypothalamic-pituitary langerhans' cell histiocytosis in female adult: A case report. ( 30633157 )
2019
18
Unusual site of Langerhans cell histiocytosis: cervical and vaginal involvement. ( 30634883 )
2019
19
Langerhans Cell Histiocytosis. ( 30661822 )
2019
20
Comparison of whole-body MRI, bone scan, and radiographic skeletal survey for lesion detection and risk stratification of Langerhans Cell Histiocytosis. ( 30670752 )
2019
21
Hearing recovery following repair of otic capsule defect secondary to semicircular canal erosion from Langerhans cell histiocytosis. ( 30690845 )
2019
22
Outcome of children with Langerhans cell histiocytosis and single-system involvement: A retrospective study at a single center in Shanghai, China. ( 30693828 )
2019
23
Langerhans cell histiocytosis isolated to the thymus in a 7-month-old infant. ( 30767312 )
2019
24
Intralesional Triamcinolone for Treating Mandibular Langerhans Cell Histiocytosis: A Case Report and Literature Review. ( 30787858 )
2019
25
Pulmonary Langerhans Cell Histiocytosis: A not so Benign Lesion. ( 30789847 )
2019
26
Zoledronic acid for relapsed Langerhans cell histiocytosis with isolated skull bone lesion. ( 30793439 )
2019
27
BRAF gene mutations in synchronous papillary thyroid carcinoma and Langerhans cell histiocytosis co-existing in the thyroid gland: a case report and literature review. ( 30795755 )
2019
28
Langerhans Cell Histiocytosis Masquerading as Hypereosinophilia in a Child. ( 30807396 )
2019
29
Secondary aneurysmal bone cyst in Langerhans cell histiocytosis: Case report, literature review. ( 30809556 )
2019
30
Agminated Spitzoid Nevi after Remission of Langerhans Cell Histiocytosis: First Italian Case and Literature Review. ( 30809687 )
2019
31
Gastrointestinal: Extensive abdominal involvement as an initial presentation of Langerhans' cell histiocytosis. ( 30811672 )
2019
32
Remission of Congenital Multi-system Type Langerhans Cell Histiocytosis with Chemotherapy. ( 30820055 )
2019
33
Diverse cutaneous manifestation of Langerhans cell histiocytosis: a 10-year retrospective cohort study. ( 30826864 )
2019
34
Langerhans Cell Histiocytosis with Multiple Fluid-fluid Levels in the Parietal Bone. ( 30828044 )
2019
35
Diffuse Subcutaneous and Muscular Langerhans Cell Histiocytosis on FDG PET/CT. ( 30829872 )
2019
36
Characteristic Reconstitution of the Spinal Langerhans Cell Histiocytosis in Young Children. ( 30839484 )
2019
37
Pediatric Langerhans cell histiocytosis: state of the science and future directions. ( 30845115 )
2019
38
Reconstruction of a severe mandibular pathological fracture caused by Langerhans cell histiocytosis using a free fibula osteocutaneous flap:a case report. ( 29372175 )
2018
39
An update on the treatment of pediatric-onset Langerhans cell histiocytosis through pharmacotherapy. ( 29355432 )
2018
40
The use of BRAF V600E mutation-specific immunohistochemistry in pediatric Langerhans cell histiocytosis. ( 28219109 )
2018
41
A Rare Case of Erdheim-Chester Disease (Non-Langerhans Cell Histiocytosis) with Concurrent Langerhans Cell Histiocytosis: A Diagnostic and Therapeutic Challenge. ( 29888013 )
2018
42
Langerhans cell histiocytosis mimicking molluscum contagiosum: A case series. ( 29528195 )
2018
43
Oral manifestation of Langerhans cell histiocytosis: a case report. ( 29884166 )
2018
44
Reflectance Confocal Microscopy for the Diagnosis of Langerhans Cell Histiocytosis. ( 29341062 )
2018
45
A case report of orbital Langerhans cell histiocytosis presenting as a orbital cellulitis. ( 29643005 )
2018
46
Classic Hodgkin lymphoma with osseous involvement mimicking Langerhans cell histiocytosis in a child. ( 29307624 )
2018
47
Rosai-Dorfman Disease: Rare Pulmonary Involvement Mimicking Pulmonary Langerhans Cell Histiocytosis and Review of the Literature. ( 29850355 )
2018
48
Outcome After Radiation Therapy for Langerhans Cell Histiocytosis Is Dependent on Site of Involvement. ( 29413280 )
2018
49
Bone metabolism in Langerhans cell histiocytosis. ( 29967185 )
2018
50
Langerhans cell histiocytosis of the orbit: A study of eight cases. ( 29930447 )
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:

38
# Name Kegg Source Accession
1 MAPK signaling pathway hsa04010

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

(show all 40)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.52 BRAF CSF1 CSF2 IL17A MAP2K1 NRAS
2
Show member pathways
13.33 BRAF CD101 CD1A CD207 CSF1 CSF2
3
Show member pathways
12.87 BRAF CSF1 CSF2 F13A1 IL17A MAP2K1
4 12.64 BRAF CSF1 MAP2K1 NRAS
5
Show member pathways
12.64 BRAF MAP2K1 NRAS TNFSF11
6
Show member pathways
12.6 BRAF CSF1 MAP2K1 NRAS
7
Show member pathways
12.34 BRAF CSF2 MAP2K1 NRAS
8
Show member pathways
12.25 BRAF MAP2K1 NRAS TNFSF11
9
Show member pathways
12.25 CSF2 IL17A MAP2K1 NRAS
10
Show member pathways
12.07 BRAF MAP2K1 NRAS
11
Show member pathways
12.05 BRAF MAP2K1 NRAS
12
Show member pathways
12.02 BRAF MAP2K1 NRAS
13
Show member pathways
11.99 BRAF MAP2K1 NRAS TNFSF11
14
Show member pathways
11.96 BRAF MAP2K1 NRAS
15
Show member pathways
11.95 BRAF MAP2K1 NRAS
16 11.91 CSF1 MAP2K1 TNFSF11
17 11.86 CD163 CSF1 CSF2
18 11.85 BRAF MAP2K1 NRAS
19 11.84 BRAF MAP2K1 NRAS
20 11.83 BRAF MAP2K1 NRAS
21 11.82 CSF1 CSF2 MAP2K1
22 11.79 BRAF MAP2K1 TNFSF11
23 11.74 CD1A CSF1 CSF2
24
Show member pathways
11.65 BRAF MAP2K1 NRAS
25 11.56 BRAF MAP2K1 NRAS
26 11.54 CD163 CSF2 IL17A
27 11.52 CSF1 CSF2 IL17A TNFSF11
28
Show member pathways
11.47 BRAF MAP2K1 NRAS
29 11.41 BRAF MAP2K1 NRAS
30 11.18 BRAF MAP2K1 NRAS
31
Show member pathways
11.17 BRAF MAP2K1
32 11.14 CSF1 CSF2
33 11.03 BRAF MAP2K1
34 11.03 BRAF MAP2K1 NRAS
35 10.95 MAP2K1 NRAS
36 10.94 CSF1 CSF2 IL17A TNFSF11
37 10.93 BRAF MAP2K1
38 10.89 CD163 CD1A CD207 CSF1 CSF2
39 10.8 CSF1 CSF2 IL17A MAP2K1 TNFSF11
40 10.71 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 region GO:0005576 9.17 CD163 CSF1 CSF2 F13A1 IL17A S100B

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

(show all 15)
# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.84 CD1A CSF2 IL17A TNFSF11
2 regulation of signaling receptor activity GO:0010469 9.78 CSF1 CSF2 IL17A TNFSF11
3 peptidyl-tyrosine phosphorylation GO:0018108 9.69 BRAF CSF2 MAP2K1
4 MAPK cascade GO:0000165 9.67 BRAF CSF2 MAP2K1 NRAS
5 positive regulation of gene expression GO:0010628 9.65 BRAF CSF1 CSF2 MAP2K1 TNFSF11
6 osteoclast differentiation GO:0030316 9.55 CSF1 TNFSF11
7 ERK1 and ERK2 cascade GO:0070371 9.52 MAP2K1 TNFSF11
8 thyroid gland development GO:0030878 9.49 BRAF MAP2K1
9 face development GO:0060324 9.46 BRAF MAP2K1
10 positive regulation of macrophage derived foam cell differentiation GO:0010744 9.43 CSF1 CSF2
11 regulation of axon regeneration GO:0048679 9.4 BRAF MAP2K1
12 cytokine-mediated signaling pathway GO:0019221 9.35 CSF1 CSF2 F13A1 IL17A TNFSF11
13 positive regulation of interleukin-23 production GO:0032747 9.32 CSF2 IL17A
14 osteoclast proliferation GO:0002158 9.16 CSF1 TNFSF11
15 positive regulation of osteoclast differentiation GO:0045672 8.8 CSF1 IL17A TNFSF11

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

# Name GO ID Score Top Affiliating Genes
1 protein tyrosine kinase activity GO:0004713 9.13 BRAF CSF2 MAP2K1
2 cytokine activity GO:0005125 8.92 CSF1 CSF2 IL17A TNFSF11

Sources for Langerhans Cell Histiocytosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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