MCID: LNG108
MIFTS: 63

Langerhans Cell Histiocytosis

Categories: Rare diseases, Immune diseases, Genetic diseases

Aliases & Classifications for Langerhans Cell Histiocytosis

MalaCards integrated aliases for Langerhans Cell Histiocytosis:

Name: Langerhans Cell Histiocytosis 57 76 53 25 37 55
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:



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 eosinophilic granuloma and non-langerhans-cell histiocytosis, 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 PEDF Induced Signaling. The drugs Cyclophosphamide and Busulfan 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

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)

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.

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

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 261)
# Related Disease Score Top Affiliating Genes
1 eosinophilic granuloma 34.4 BRAF CD1A
2 non-langerhans-cell histiocytosis 33.4 BRAF CD163 CD207 F13A1
3 langerhans cell sarcoma 32.4 CD163 CD1A CD207
4 juvenile xanthogranuloma 32.3 BRAF F13A1 S100B
5 erdheim-chester disease 32.2 BRAF NRAS
6 dendritic cell tumor 32.0 CD1A CD207 IFNG S100B
7 histiocytosis 30.3 BRAF CD101 CD163 CD1A CD207 CSF2
8 periodontitis 30.3 IFNG IL17A TNFSF11
9 generalized eruptive histiocytosis 30.1 F13A1 S100B
10 rosai-dorfman disease 30.0 CD163 MAP2K1 S100B
11 fibroblastic rheumatism 29.8 CD163 F13A1
12 monocytic leukemia 29.6 CSF1 CSF2 IFNG
13 fibrous histiocytoma 29.2 CD163 F13A1 S100B
14 juvenile myelomonocytic leukemia 28.9 BRAF CSF2 NRAS
15 myeloma, multiple 28.5 BRAF CSF2 NRAS TNFSF11
16 adult pulmonary langerhans cell histiocytosis 12.5
17 hashimoto-pritzker syndrome 12.3
18 letterer-siwe disease 12.1
19 leydig cell hypoplasia 11.8
20 lissencephaly with cerebellar hypoplasia 11.8
21 interstitial lung disease 11.4
22 dentin dysplasia, type i 11.1
23 histiocytosis, progressive mucinous 11.1
24 infantile myofibromatosis 11.1
25 reticulohistiocytic granuloma 11.1
26 thyroiditis 10.5
27 lipogranulomatosis 10.5
28 diabetes insipidus 10.5
29 myopericytoma 10.5 BRAF S100B
30 myofibroma 10.4 CD1A F13A1
31 lymphoma 10.4
32 opportunistic mycosis 10.4 CD101 IFNG IL17A
33 syringocystadenoma papilliferum 10.4 BRAF S100B
34 cavernous sinus meningioma 10.4 CD163 CD207
35 leukemia 10.4
36 alk+ histiocytosis 10.3 CD1A CD207 F13A1
37 candida glabrata 10.3 CSF2 IFNG
38 trichosporonosis 10.3 CSF2 IFNG IL17A
39 carotid artery thrombosis 10.3 CSF2 IFNG
40 cervicitis 10.3
41 respiratory system disease 10.2 CSF2 IFNG IL17A
42 mandibular cancer 10.2 CSF1 TNFSF11
43 thyroid cancer, nonmedullary, 1 10.2
44 pneumothorax 10.2
45 spondyloarthropathy 10.2 IFNG IL17A TNFSF11
46 histiocytic and dendritic cell cancer 10.2 CD163 CD207
47 immune system disease 10.2 CSF2 IFNG IL17A
48 bone inflammation disease 10.2 IFNG IL17A TNFSF11
49 sarcoma 10.2
50 human t-cell leukemia virus type 2 10.2 CSF2 IFNG

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 14)
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.26 BRAF CD101 CD163 CD207 CSF1 CSF2
2 homeostasis/metabolism MP:0005376 10.23 BRAF CD163 CSF1 CSF2 F13A1 IFNG
3 cardiovascular system MP:0005385 10.19 BRAF CD163 CSF1 CSF2 F13A1 IFNG
4 endocrine/exocrine gland MP:0005379 10.19 BRAF CSF1 CSF2 F13A1 IFNG IL17A
5 immune system MP:0005387 10.17 BRAF CD101 CD163 CD207 CSF1 CSF2
6 craniofacial MP:0005382 10.13 BRAF CSF1 CSF2 IFNG IL17A MAP2K1
7 mortality/aging MP:0010768 10.07 BRAF CSF1 CSF2 F13A1 IFNG IL17A
8 integument MP:0010771 10.06 LIF MAP2K1 NRAS S100B TNFSF11 BRAF
9 digestive/alimentary MP:0005381 9.99 IFNG IL17A LIF MAP2K1 NRAS BRAF
10 reproductive system MP:0005389 9.86 CSF2 F13A1 IFNG LIF MAP2K1 TNFSF11
11 respiratory system MP:0005388 9.81 CSF2 F13A1 IFNG IL17A LIF TNFSF11
12 neoplasm MP:0002006 9.8 BRAF CSF2 IFNG MAP2K1 NRAS TNFSF11
13 skeleton MP:0005390 9.61 BRAF CSF1 CSF2 IFNG IL17A LIF
14 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 120)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cyclophosphamide Approved, Investigational Phase 2, Phase 3,Phase 1,Not Applicable 50-18-0, 6055-19-2 2907
2
Busulfan Approved, Investigational Phase 2, Phase 3,Phase 1,Early Phase 1,Not Applicable 55-98-1 2478
3
Prednisolone Approved, Vet_approved Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable 50-24-8 5755
4
Methylprednisolone Approved, Vet_approved Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable 83-43-2 6741
5
Vinblastine Approved Phase 3,Phase 2,Not Applicable 865-21-4 13342 241903
6
Methotrexate Approved Phase 3,Phase 2,Not Applicable 1959-05-2, 59-05-2 126941
7
Prednisone Approved, Vet_approved Phase 3,Phase 2,Not Applicable 53-03-2 5865
8
Vincristine Approved, Investigational Phase 2, Phase 3 2068-78-2, 57-22-7 5978
9
Cytarabine Approved, Investigational Phase 2, Phase 3,Phase 3 147-94-4 6253
10
Indomethacin Approved, Investigational Phase 2, Phase 3 53-86-1 3715
11
Cladribine Approved, Investigational Phase 2, Phase 3 4291-63-8 20279
12
leucovorin Approved, Nutraceutical Phase 3,Phase 2,Not Applicable 58-05-9 143 6006
13
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3,Phase 2,Not Applicable 59-30-3 6037
14 Prednisolone acetate Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
15 Alkylating Agents Phase 2, Phase 3,Phase 1,Not Applicable
16 glucocorticoids Phase 3,Phase 2,Phase 1,Not Applicable
17 Methylprednisolone acetate Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
18 Methylprednisolone Hemisuccinate Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
19 Hormone Antagonists Phase 3,Phase 2,Phase 1,Not Applicable
20 Hormones Phase 3,Phase 2,Phase 1,Not Applicable
21 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3,Phase 2,Phase 1,Not Applicable
22 Peripheral Nervous System Agents Phase 2, Phase 3,Phase 1,Not Applicable
23 Immunosuppressive Agents Phase 3,Phase 2,Phase 1,Not Applicable
24 Prednisolone hemisuccinate Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
25 Prednisolone phosphate Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
26 Anti-Inflammatory Agents Phase 3,Phase 2,Phase 1,Not Applicable
27 Antirheumatic Agents Phase 3,Phase 2,Phase 1,Not Applicable
28 Antimetabolites Phase 3,Phase 2,Phase 1,Not Applicable
29 Antimetabolites, Antineoplastic Phase 3,Phase 2,Phase 1,Not Applicable
30 Antineoplastic Agents, Alkylating Phase 2, Phase 3,Phase 1,Not Applicable
31 Antineoplastic Agents, Hormonal Phase 3,Phase 2,Phase 1,Not Applicable
32 Analgesics Phase 2, Phase 3
33 6-Mercaptopurine Phase 3,Phase 2
34 Rho(D) Immune Globulin Phase 2, Phase 3
35 Folic Acid Antagonists Phase 3,Phase 2,Not Applicable
36 gamma-Globulins Phase 2, Phase 3
37 Analgesics, Non-Narcotic Phase 2, Phase 3
38 Dermatologic Agents Phase 3,Phase 2,Not Applicable
39 Nucleic Acid Synthesis Inhibitors Phase 3,Phase 2,Not Applicable
40 Vitamin B Complex Phase 3,Phase 2,Not Applicable
41 Antibodies Phase 2, Phase 3
42 Immunoglobulins Phase 2, Phase 3
43 Immunoglobulins, Intravenous Phase 2, Phase 3
44 Anti-Infective Agents Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
45 Anti-Inflammatory Agents, Non-Steroidal Phase 2, Phase 3
46 Antiviral Agents Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
47 Antimitotic Agents Phase 3,Phase 2,Not Applicable
48 Antineoplastic Agents, Phytogenic Phase 3,Phase 2,Not Applicable
49 2-chloro-3'-deoxyadenosine Phase 2, Phase 3
50 Cyclooxygenase Inhibitors Phase 2, Phase 3

Interventional clinical trials:

(show all 48)
# Name Status NCT ID Phase Drugs
1 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
2 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
3 Vinblastine/Prednisone Versus Single Therapy With Cytarabine for Langerhans Cell Histiocytosis (LCH) Recruiting NCT02670707 Phase 3 Cytarabine;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 A Study to Test the Ability of and Safety of GSK2110183 in Treating Langerhans Cell Histiocytosis Completed NCT01395004 Phase 2 GSK2110183
8 Treatment of Resistant Langerhans Cell Histiocytosis With ENBREL Completed NCT00048373 Phase 2 Etanercept
9 Study of Sequential Administration of Oral 6-Thioguanine After Methotrexate in Patients With LCH Completed NCT00588536 Phase 2 Methotrexate;6-Thioguanine;Leucovorin Calcium
10 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)
11 Deferasirox in Treating Iron Overload Caused By Blood Transfusions in Patients With Hematologic Malignancies Completed NCT01273766 Phase 2 deferasirox
12 A Study of Vemurafenib in Participants With BRAF V600 Mutation-Positive Cancers Completed NCT01524978 Phase 2 cetuximab;vemurafenib;vemurafenib
13 Phase II Study of Clofarabine in Patients With Recurrent or Refractory Langerhans Cell Histiocytosis Recruiting NCT02425904 Phase 2 Clofarabine
14 Methotrexate and Cytosine in Adult Langerhans Cell Histiocytosis Recruiting NCT02389400 Phase 2 methotrexate
15 Denosumab for the Treatment of Adult LCH Recruiting NCT03270020 Phase 2 Denosumab 70 MG/ML [Xgeva]
16 Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphomas, or Histiocytic Disorders (The Pediatric MATCH Screening Trial) Recruiting NCT03155620 Phase 2 Ensartinib;Erdafitinib;Larotrectinib;Olaparib;Palbociclib;PI3K/mTOR Inhibitor LY3023414;Selumetinib Sulfate;Tazemetostat;Vemurafenib
17 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
18 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
19 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
20 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
21 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
22 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
23 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
24 Evaluation of Efficacy and Tolerance of Cladribine in Symptomatic Pulmonary Langerhans Cell Histiocytosis Active, not recruiting NCT01473797 Phase 2 Cladribine
25 A Study of Lenalidomide for Adult Histiocyte Disorders Active, not recruiting NCT02523040 Phase 2 Lenalidomide
26 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
27 Reduced Intensity Hematopoietic Cell Transplantation for Patients With Resistant Langerhans Cell Histiocytosis Terminated NCT00618540 Phase 2 fludarabine phosphate;melphalan
28 Clofarabine for Langerhans in Pedi Withdrawn NCT01796405 Phase 2 Clofarabine
29 Sunitinib Malate in Treating HIV-Positive Patients With Cancer Receiving Antiretroviral Therapy Completed NCT00890747 Phase 1 sunitinib malate
30 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
31 A Study to Determine Safety, Tolerability and Pharmacokinetics of Oral Dabrafenib In Children and Adolescent Subjects Recruiting NCT01677741 Phase 1 Dabrafenib
32 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
33 Indium In 111 Pentetreotide in Treating Patients With Refractory Cancer Terminated NCT00002947 Phase 1
34 Cardiovascular Risk Factors and LCH in Adults Unknown status NCT00483925
35 Combination Chemotherapy in Treating Young Patients With Langerhans Cell Histiocytosis Completed NCT00276757 Not Applicable leucovorin calcium;methotrexate;prednisolone;vinblastine sulfate
36 Prospective Evaluation of Adult Pulmonary Langerhans Cell Histiocytosis Completed NCT01225601
37 Evaluation of Outcome of Adult Pulmonary Langerhans Cell Histiocytosis Based on Lung HRCT and Lung Function Completed NCT01651507
38 Ceftriaxone in Non-neutropenic Fever Completed NCT01225718
39 Assessment of Safety of Air Travel in Patients With Pulmonary Langerhans Cell Histiocytosis Recruiting NCT03052101
40 Characterization of Pulmonary Langerhans Cell Histiocytosis Recruiting NCT03093727
41 Evaluation of Exercise Capacity and Exercise Limitation in Patients With Pulmonary Langerhans Cell Histiocytosis Recruiting NCT02665546
42 A Study of Memory, Thinking, and Brain Imaging in Adults With Histiocytosis Recruiting NCT03127709
43 Uptake and Biodistribution of 18F-fluorocholine in Histiocytic Disorders by PET Imaging and Biopsy Measurement Recruiting NCT02608619
44 CD34+ (Non-Malignant) Stem Cell Selection for Patients Receiving Allogeneic Stem Cell Transplantation Recruiting NCT01966367 Early Phase 1
45 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
46 Collecting and Storing Tissue From Young Patients With Cancer Recruiting NCT00898755
47 Blood Stem Cell Transplant in Treating Patients With Hematologic Cancer Terminated NCT00008216
48 Allogeneic Bone Marrow Transplantation in Patients With Primary Immunodeficiencies Terminated NCT00006054 Not Applicable anti-thymocyte globulin;busulfan;cyclophosphamide;cyclosporine;etoposide;methotrexate;methylprednisolone;prednisone

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, Spleen, Bone Marrow, Thyroid, Lung

Publications for Langerhans Cell Histiocytosis

Articles related to Langerhans Cell Histiocytosis:

(show top 50) (show all 1014)
# Title Authors Year
1
Reconstruction of a severe mandibular pathological fracture caused by Langerhans cell histiocytosis using a free fibula osteocutaneous flap:a case report. ( 29372175 )
2018
2
An update on the treatment of pediatric-onset Langerhans cell histiocytosis through pharmacotherapy. ( 29355432 )
2018
3
The use of BRAF V600E mutation-specific immunohistochemistry in pediatric Langerhans cell histiocytosis. ( 28219109 )
2018
4
A Rare Case of Erdheim-Chester Disease (Non-Langerhans Cell Histiocytosis) with Concurrent Langerhans Cell Histiocytosis: A Diagnostic and Therapeutic Challenge. ( 29888013 )
2018
5
Langerhans cell histiocytosis mimicking molluscum contagiosum: A case series. ( 29528195 )
2018
6
Oral manifestation of Langerhans cell histiocytosis: a case report. ( 29884166 )
2018
7
Reflectance Confocal Microscopy for the Diagnosis of Langerhans Cell Histiocytosis. ( 29341062 )
2018
8
A case report of orbital Langerhans cell histiocytosis presenting as a orbital cellulitis. ( 29643005 )
2018
9
Classic Hodgkin lymphoma with osseous involvement mimicking Langerhans cell histiocytosis in a child. ( 29307624 )
2018
10
Rosai-Dorfman Disease: Rare Pulmonary Involvement Mimicking Pulmonary Langerhans Cell Histiocytosis and Review of the Literature. ( 29850355 )
2018
11
Outcome After Radiation Therapy for Langerhans Cell Histiocytosis Is Dependent on Site of Involvement. ( 29413280 )
2018
12
Bone metabolism in Langerhans cell histiocytosis. ( 29967185 )
2018
13
Langerhans cell histiocytosis of the orbit: A study of eight cases. ( 29930447 )
2018
14
National Jewish Health Classical Pulmonary Radiology Case Reports: Imaging Features of Langerhans Cell Histiocytosis. ( 29878226 )
2018
15
Radiotherapy for Langerhans cell histiocytosis with paraplegia: A rare oncologic emergency case report in infancy and literature review. ( 29907475 )
2018
16
Congenital Langerhans cell histiocytosis presenting in a 27-week-gestation neonate. ( 29436047 )
2018
17
MRI features in solitary cerebral Langerhans cell histiocytosis : a case report and review of literature. ( 29929024 )
2018
18
Langerhans cell histiocytosis in an 18-month-old child presenting as periorbital cellulitis. ( 29755272 )
2018
19
Langerhans cell histiocytosis of bone in an adult: A case report. ( 29904462 )
2018
20
Intraoperative MRI-guided biopsy in the diagnosis of suprasellar Langerhans Cell Histiocytosis. ( 29317365 )
2018
21
Immune Thrombocytopenia in a Child With Refractory Langerhans Cell Histiocytosis Following Cladribine Containing Therapy. ( 29889804 )
2018
22
Pulmonary Langerhans cell histiocytosis, acute myeloid leukemia, and myelofibrosis in a large family and review of the literature. ( 29428448 )
2018
23
Pulmonary Langerhans Cell Histiocytosis(PLCH) masquerading as Atypical Mycobacterial infection. ( 29942736 )
2018
24
Langerhans Cell Histiocytosis of the Adult Cervical Spine: A Case Report and Literature Review. ( 29890538 )
2018
25
Localised Langerhans cell histiocytosis of the hypothalamic-pituitary region: case report and literature review. ( 29858859 )
2018
26
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
27
RAF/MEK/extracellular signal-related kinase pathway suppresses dendritic cell migration and traps dendritic cells in Langerhans cell histiocytosis lesions. ( 29263218 )
2018
28
A unique manifestation of Langerhans cell histiocytosis: Diagnostic and therapeutic considerations of atypical cases. ( 29940687 )
2018
29
Isolated Langerhans Cell Histiocytosis of Orbit: A Case Report and Review of the Literature. ( 29850325 )
2018
30
Juvenile Xanthogranuloma in a Pediatric Patient with Langerhans Cell Histiocytosis. ( 29765943 )
2018
31
A case of adult onset folliculocentric Langerhans cell histiocytosis. ( 29924409 )
2018
32
Successful management of multiple-systemic Langerhans cell histiocytosis involving endocrine organs in an adult: A case report and review of literature. ( 29952977 )
2018
33
Cutaneous Langerhans cell histiocytosis with gastrointestinal involvement treated with dabrafenib. ( 29379859 )
2018
34
A potentially important role for T cells and regulatory T cells in Langerhans cell histiocytosis. ( 29928999 )
2018
35
Disseminated juvenile xanthogranuloma occurring after treatment of Langerhans cell histiocytosis: a case report. ( 29209883 )
2017
36
Adult Langerhans cell histiocytosis with pulmonary and colorectoanal involvement: a case report. ( 28942734 )
2017
37
Congenital Self Healing Langerhans Cell Histiocytosis. ( 28929464 )
2017
38
Langerhans cell histiocytosis in a patient with hairy cell leukemia: a tale of divergence. ( 28302693 )
2017
39
Langerhans' Cell Histiocytosis Mimicking a Pott Puffy Tumor. ( 28562520 )
2017
40
Vinblastine chemotherapy in adult patients with langerhans cell histiocytosis: a multicenter retrospective study. ( 28532436 )
2017
41
Low periostin levels in adult patients with Langerhans cell histiocytosis are independently associated with the disease activity. ( 28521873 )
2017
42
Pulmonary langerhans cell histiocytosis - insight into the incidence of alfa-1-antitrypsin (A1ATD) deficiency alleles. ( 29288477 )
2017
43
Langerhans cell histiocytosis in an adult female presenting with widespread confluent crusted papules and review of adult cases confined to skin. ( 28880370 )
2017
44
Clinical course of the bony lesion of single-system single-site Langerhans cell histiocytosis - Is appropriate follow-up sufficient treatment? ( 28927957 )
2017
45
Dermoscopy of Langerhans cell histiocytosis. ( 28087041 )
2017
46
Cerebellar Disease Mimicking Cerebrotendinous Xanthomatosis: Langerhans Cell Histiocytosis. ( 28554492 )
2017
47
Killer cell immunoglobulin-like receptor 2DL4 is expressed in and suppresses the cell growth of Langerhans cell histiocytosis. ( 28445138 )
2017
48
Atlantoaxial Langerhans cell histiocytosis radiographic characteristics and corresponding prognosis analysis. ( 29021671 )
2017
49
Hematopoietic origin of Langerhans cell histiocytosis and Erdheim Chester disease in adults. ( 28512190 )
2017
50
Role of<sup>18</sup>F-FDG PET/CT in patients affected by Langerhans cell histiocytosis. ( 28748503 )
2017

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 43)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.49 BRAF CSF1 CSF2 IFNG IL17A LIF
2
Show member pathways
13.4 BRAF CD101 CD1A CD207 CSF1 CSF2
3
Show member pathways
13.31 CSF1 CSF2 IL17A LIF NRAS TNFSF11
4
Show member pathways
13.17 CSF1 CSF2 IL17A LIF NRAS TNFSF11
5
Show member pathways
12.96 BRAF CSF1 CSF2 F13A1 IFNG IL17A
6
Show member pathways
12.55 BRAF IFNG MAP2K1 NRAS
7
Show member pathways
12.5 CSF2 IFNG IL17A LIF
8
Show member pathways
12.31 BRAF CSF2 IFNG MAP2K1 NRAS
9
Show member pathways
12.3 BRAF MAP2K1 NRAS TNFSF11
10
Show member pathways
12.13 CSF2 IFNG IL17A MAP2K1 NRAS
11
Show member pathways
12.12 BRAF MAP2K1 NRAS
12
Show member pathways
12.05 BRAF MAP2K1 NRAS
13 11.99 LIF MAP2K1 NRAS
14
Show member pathways
11.99 BRAF MAP2K1 NRAS
15
Show member pathways
11.98 BRAF MAP2K1 NRAS
16
Show member pathways
11.98 IFNG IL17A MAP2K1 NRAS
17 11.89 CSF1 IFNG MAP2K1 TNFSF11
18 11.88 F13A1 IL17A LIF
19 11.88 BRAF MAP2K1 NRAS
20 11.87 BRAF MAP2K1 NRAS
21 11.86 BRAF MAP2K1 NRAS
22 11.83 BRAF MAP2K1 TNFSF11
23 11.82 BRAF IFNG MAP2K1
24 11.8 IFNG MAP2K1 NRAS
25 11.77 CD1A CSF1 CSF2
26 11.77 CD163 CSF1 CSF2 IFNG
27 11.7 CSF1 CSF2 LIF MAP2K1
28
Show member pathways
11.69 BRAF MAP2K1 NRAS
29 11.66 CD101 IFNG IL17A
30 11.62 BRAF MAP2K1 NRAS
31 11.61 CSF1 CSF2 IFNG IL17A TNFSF11
32
Show member pathways
11.56 BRAF MAP2K1 NRAS
33
Show member pathways
11.52 BRAF IFNG MAP2K1
34 11.5 BRAF MAP2K1 NRAS
35 11.4 CSF1 CSF2 LIF
36 11.32 BRAF MAP2K1 NRAS
37 11.22 BRAF MAP2K1 NRAS
38 11.11 CSF1 CSF2 IFNG
39 11.04 BRAF MAP2K1
40 10.97 MAP2K1 NRAS
41 10.97 CD163 CD1A CD207 CSF1 CSF2 IFNG
42 10.94 BRAF MAP2K1
43 10.73 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 9.88 CSF1 CSF2 IFNG LIF S100B
2 immune response GO:0006955 9.85 CD1A CSF2 IFNG IL17A LIF TNFSF11
3 MAPK cascade GO:0000165 9.8 BRAF CSF2 MAP2K1 NRAS
4 regulation of signaling receptor activity GO:0010469 9.8 CSF1 CSF2 IFNG IL17A LIF TNFSF11
5 positive regulation of gene expression GO:0010628 9.73 BRAF CSF1 CSF2 IFNG LIF MAP2K1
6 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.63 CSF2 IFNG LIF
7 cytokine-mediated signaling pathway GO:0019221 9.63 CSF1 CSF2 F13A1 IL17A LIF TNFSF11
8 positive regulation of peptidyl-serine phosphorylation of STAT protein GO:0033141 9.55 IFNG LIF
9 face development GO:0060324 9.54 BRAF MAP2K1
10 positive regulation of macrophage derived foam cell differentiation GO:0010744 9.52 CSF1 CSF2
11 positive regulation of macrophage differentiation GO:0045651 9.51 CSF1 LIF
12 regulation of axon regeneration GO:0048679 9.43 BRAF MAP2K1
13 osteoclast proliferation GO:0002158 9.4 CSF1 TNFSF11
14 positive regulation of interleukin-23 production GO:0032747 9.13 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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