LCH
MCID: LNG108
MIFTS: 57

Langerhans Cell Histiocytosis (LCH)

Categories: Blood diseases, Bone diseases, Cancer diseases, Immune diseases, Rare diseases, Respiratory diseases
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Aliases & Classifications for Langerhans Cell Histiocytosis

MalaCards integrated aliases for Langerhans Cell Histiocytosis:

Name: Langerhans Cell Histiocytosis 57 19 42 58 75 28 53 14 16 33
Histiocytosis X 11 19 42 58 75 53 33
Langerhans Cell Granulomatosis 11 42 58
Lch 57 19 42
Histiocytosis, Langerhans-Cell 43 71
Langerhans-Cell Histiocytosis 11 14
Hashimoto-Pritzger Disease 42 71
Letterer-Siwe Disease 11 71
Letterer-Siwe Disease Involving Lymph Nodes of Inguinal Region and Lower Limb 11
Letterer-Siwe Disease of Lymph Nodes of Inguinal Region Amd/or Lower Limb 11
Letterer-Siwe Disease of Lymph Nodes of Inguinal Region and/or Lower Limb 11
Letterer-Siwe Disease of Lymph Nodes of Inguinal Region and Lower Limb 11
Letterer-Siwe Disease Involving Lymph Nodes of Axilla and Upper Limb 11
Letterer-Siwe Disease Involving Lymph Nodes of Head, Face, and Neck 11
Letterer-Siwe Disease Involving Lymph Nodes of Head, Face and Neck 11
Letterer-Siwe Disease of Lymph Nodes of Axilla and/or Upper Limb 11
Letterer-Siwe Disease of Lymph Nodes of Head, Face and/or Neck 11
Letterer-Siwe Disease Involving Lymph Nodes of Multiple Sites 11
Letterer-Siwe Disease of Lymph Nodes of Axilla and Upper Limb 11
Letterer-Siwe Disease Involving Intra-Abdominal Lymph Nodes 11
Letterer-Siwe Disease of Lymph Nodes of Head, Face and Neck 11
Letterer-Siwe Disease Involving Intrathoracic Lymph Nodes 11
Letterer-Siwe Disease Involving Intrapelvic Lymph Nodes 11
Letterer-Siwe Disease of Lymph Nodes of Multiple Sites 11
Letterer-Siwe Disease of Intra-Abdominal Lymph Nodes 11
Letterer-Siwe Disease of Intrathoracic Lymph Nodes 11
Letterer-Siwe Disease of Intrapelvic Lymph Nodes 11
Letterer-Siwe Disease Involving Spleen 11
Langerhans-Cell Histiocytosis Nos 33
Letterer-Siwe Disease of Spleen 11
Langerhan's Cell Histiocytosis 11
Familial Letterer-Siwe Disease 43

Characteristics:


Prevelance:

1-9/100000 (Europe) 58

Age Of Onset:

All ages 58

Classifications:

Orphanet: 58  
Rare respiratory diseases
Rare systemic and rhumatological diseases
Rare haematological diseases


External Ids:

Disease Ontology 11 DOID:2571
OMIM® 57 604856
ICD9CM 34 202.5
NCIt 49 C3107 C3160
SNOMED-CT 68 154583006 234439008
ICD10 31 C96.0 C96.6
MESH via Orphanet 44 D006646
ICD10 via Orphanet 32 C96.0 C96.5 C96.6
UMLS via Orphanet 72 C0019621
Orphanet 58 ORPHA389
MedGen 40 C0019621
UMLS 71 C0019621 C0023381 C3661439

Summaries for Langerhans Cell Histiocytosis

MedlinePlus Genetics: 42 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.In approximately 80 percent of affected individuals, one or more granulomas develop in the bones, causing pain and swelling. The granulomas, which usually occur in the skull or the long bones of the arms or legs, may cause the bone to fracture.Granulomas also frequently occur in the skin, appearing as blisters, reddish bumps, or rashes which can be mild to severe. The pituitary gland may also be affected; this gland is located at the base of the brain and produces hormones that control many important body functions. Without hormone supplementation, affected individuals may experience delayed or absent puberty or an inability to have children (infertility). In addition, pituitary gland damage may result in the production of excessive amounts of urine (diabetes insipidus) and dysfunction of another gland called the thyroid. Thyroid dysfunction can affect the rate of chemical reactions in the body (metabolism), body temperature, skin and hair texture, and behavior.In 15 to 20 percent of cases, Langerhans cell histiocytosis affects the lungs, liver, or blood-forming (hematopoietic) system; damage to these organs and tissues may be life-threatening. Lung involvement, which appears as swelling of the small airways (bronchioles) and blood vessels of the lungs, results in stiffening of the lung tissue, breathing problems, and increased risk of infection. Hematopoietic involvement, which occurs when the Langerhans cells crowd out blood-forming cells in the bone marrow, leads to a general reduction in the number of blood cells (pancytopenia). Pancytopenia results in fatigue due to low numbers of red blood cells (anemia), frequent infections due to low numbers of white blood cells (neutropenia), and clotting problems due to low numbers of platelets (thrombocytopenia).Other signs and symptoms that may occur in Langerhans cell histiocytosis, depending on which organs and tissues have Langerhans cell deposits, include swollen lymph nodes, abdominal pain, yellowing of the skin and whites of the eyes (jaundice), delayed puberty, protruding eyes, dizziness, irritability, and seizures. About 1 in 50 affected individuals experience deterioration of neurological function (neurodegeneration).Langerhans cell histiocytosis is often diagnosed in childhood, usually between ages 2 and 3, but can appear at any age. Most individuals with adult-onset Langerhans cell histiocytosis are current or past smokers; in about two-thirds of adult-onset cases the disorder affects only the lungs.The severity of Langerhans cell histiocytosis, and its signs and symptoms, vary widely among affected individuals. Certain presentations or forms of the disorder were formerly considered to be separate diseases. Older names that were sometimes used for forms of Langerhans cell histiocytosis include eosinophilic granuloma, Hand-Schüller-Christian disease, and Letterer-Siwe disease.In many people with Langerhans cell histiocytosis, the disorder eventually goes away with appropriate treatment. It may even disappear on its own, especially if the disease occurs only in the skin. However, some complications of the condition, such as diabetes insipidus or other effects of tissue and organ damage, may be permanent.

MalaCards based summary: Langerhans Cell Histiocytosis, also known as histiocytosis x, is related to histiocytosis and non-langerhans-cell histiocytosis, and has symptoms including fever An important gene associated with Langerhans Cell Histiocytosis is MAP2K1 (Mitogen-Activated Protein Kinase Kinase 1), and among its related pathways/superpathways are MIF Mediated Glucocorticoid Regulation and Innate Immune System. The drugs Prednisone and Mycophenolic acid have been mentioned in the context of this disorder. Affiliated tissues include spleen, bone marrow and bone, and related phenotypes are immune system and hematopoietic system

GARD: 19 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 genetic changes 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.

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) (Updated 08-Dec-2022)

Orphanet: 58 Langerhans cell histiocytosis (LCH) is a systemic disease associated with the proliferation and accumulation (usually in granulomas) of Langerhans cells in various tissues.

Disease Ontology: 11 A histiocytosis that is characterized by clonal proliferation of Langerhans cells.

Wikipedia: 75 Langerhans cell histiocytosis (LCH) is an abnormal clonal proliferation of Langerhans cells, abnormal... 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 963)
# Related Disease Score Top Affiliating Genes
1 histiocytosis 32.4 TNF SERPINA3 S100B NRAS MAP2K1 IL17A
2 non-langerhans-cell histiocytosis 32.4 SERPINA3 NRAS MAP2K1 CD68 CD207 CD1E
3 central diabetes insipidus 32.1 CD1E CD1C CD1B CD1A
4 langerhans cell sarcoma 32.1 CD68 CD207 CD1E CD1C CD1B CD1A
5 histiocytosis-lymphadenopathy plus syndrome 32.0 SERPINA3 S100B CD68 CD1E CD1C CD1B
6 juvenile xanthogranuloma 32.0 SERPINA3 FSCN1 F13A1 CD68 CD207 CD1E
7 multicentric reticulohistiocytosis 31.9 SERPINA3 CD68 CD1E CD1C CD1B CD1A
8 erdheim-chester disease 31.9 SERPINA3 NRAS MAP2K1 CD1E CD1C CD1B
9 exanthem 31.9 TNF IL17A CSF2
10 reticulohistiocytic granuloma 31.9 SERPINA3 CD68 CD207 CD1E CD1C CD1B
11 respiratory failure 31.4 TNF SERPINA3 IL17A CSF2
12 otorrhea 31.2 CD207 CD1E CD1C CD1B CD1A
13 cellulitis 31.2 TNF IL17A CSF2
14 sarcoidosis 1 31.2 TNF SERPINA3 IL17A CD68
15 bronchiolitis 31.1 TNF IL17A IL11 CSF2
16 fungal infectious disease 31.0 TNF IL17A CSF2
17 seborrheic dermatitis 30.9 TNF IL17A CD207 CD1E CD1C CD1B
18 interstitial pneumonitis, desquamative, familial 30.8 CD1E CD1B CD1A
19 thrombocytosis 30.8 LIF IL11 CSF2
20 crohn's disease 30.8 TNF SERPINA3 IL17A CSF2
21 castleman disease 30.8 S100B FSCN1 F13A1
22 bronchopneumonia 30.7 TNF SERPINA3 IL17A CD68
23 fibrous histiocytoma 30.7 SERPINA3 S100B F13A1 CD68
24 orbital disease 30.7 CD68 CD1E CD1C CD1B CD1A
25 nervous system disease 30.7 TNF SERPINA3 S100B MAP2K1 IL17A CSF2
26 lichen planus 30.7 TNF IL17A CD1A
27 allergic disease 30.7 TNF SERPINA3 IL17A IL11 CSF2
28 orbital cancer 30.7 CD68 CD1E CD1C CD1B CD1A
29 kimura disease 30.7 CD1E CD1B CD1A
30 contact dermatitis 30.7 TNF SERPINA3 IL17A CSF2
31 cutaneous fibrous histiocytoma 30.6 SERPINA3 S100B F13A1 CD68
32 folliculitis 30.6 TNF IL17A CD1E CD1B CD1A
33 melanoma in congenital melanocytic nevus 30.6 S100B NRAS BRAF
34 lymph node disease 30.6 TNF SERPINA3 IL17A CD68
35 immune deficiency disease 30.6 TNF SERPINA3 S100B IL17A CSF2
36 celiac disease 1 30.6 TNF SERPINA3 IL17A CSF2
37 myeloma, multiple 30.5 NRAS LIF IL11 CSF2 BRAF
38 meningioma, familial 30.5 SERPINA3 S100B CD68 CD1E CD1B CD1A
39 neutrophilic dermatosis, acute febrile 30.5 TNF SERPINA3 IL17A
40 malignant fibrous histiocytoma 30.5 SERPINA3 S100B F13A1 CD68
41 anterior uveitis 30.5 TNF SERPINA3 IL17A
42 panuveitis 30.5 TNF SERPINA3 IL17A
43 lymphoma, non-hodgkin, familial 30.5 SERPINA3 NRAS MAP2K1 CSF2 BRAF
44 lichen nitidus 30.5 CD1E CD1B CD1A
45 mixed phenotype acute leukemia, t/myeloid 30.4 CD1E CD1B CD1A
46 splenic disease 30.4 TNF SERPINA3 IL17A CSF2
47 meningitis 30.4 TNF S100B IL17A CSF2
48 skin squamous cell carcinoma 30.4 NRAS MAP2K1 BRAF
49 bone inflammation disease 30.4 TNF SERPINA3 IL17A CSF2
50 histiocytic sarcoma 30.4 SERPINA3 FSCN1 CD68 CD1E CD1C CD1B

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 (Updated 08-Dec-2022)

UMLS symptoms related to Langerhans Cell Histiocytosis:


fever

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

25
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 no effect GR00402-S-1 10.14 BRAF CD101 CD1A CD1B CD1C CD1E
2 no effect GR00402-S-2 10.14 BRAF CD101 CD1A CD1E CD68 CSF2

MGI Mouse Phenotypes related to Langerhans Cell Histiocytosis:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 10.03 BRAF CD101 CD207 CD68 CSF2 CTSE
2 hematopoietic system MP:0005397 9.77 BRAF CD101 CD207 CD68 CSF2 CTSE
3 respiratory system MP:0005388 9.76 BRAF CD207 CSF2 F13A1 FSCN1 IL17A
4 integument MP:0010771 9.4 BRAF CSF2 CTSE F13A1 FSCN1 IL17A

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
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
2
Mycophenolic acid Approved, Investigational Phase 4 24280-93-1 446541
3 Antineoplastic Agents, Hormonal Phase 4
4 Anti-Infective Agents Phase 4
5 Hormones Phase 4
6 Hormone Antagonists Phase 4
7 glucocorticoids Phase 4
8 Anti-Inflammatory Agents Phase 4
9 Anti-Bacterial Agents Phase 4
10 Antitubercular Agents Phase 4
11 Antibiotics, Antitubercular Phase 4
12
Vinblastine Approved Phase 3 865-21-4 241903 13342
13
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
14
Prednisolone acetate Approved, Vet_approved Phase 3 52-21-1
15
Prednisolone Approved, Vet_approved Phase 3 50-24-8 4894 5755
16
Mercaptopurine Approved Phase 3 50-44-2 667490
17
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5 1875
18
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 4159 6741
19
Cytarabine Approved, Investigational Phase 3 147-94-4 6253
20
Levoleucovorin Approved, Experimental, Investigational Phase 2, Phase 3 68538-85-2, 58-05-9, 73951-54-9 149436 6006
21
Methotrexate Approved Phase 2, Phase 3 1959-05-2, 59-05-2 4112 126941
22
Vincristine Approved, Investigational Phase 3 2068-78-2, 57-22-7 5978
23
Cladribine Approved, Investigational Phase 2, Phase 3 4291-63-8 20279
24
Indomethacin Approved, Investigational Phase 2, Phase 3 53-86-1 3715
25
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
26
Busulfan Approved, Investigational Phase 2, Phase 3 55-98-1 2478
27
Folic acid Approved, Nutraceutical, Vet_approved Phase 2, Phase 3 59-30-3 6037
28
Prednisolone hemisuccinate Experimental Phase 3 2920-86-7 4897
29 Vitamins Phase 3
30 Trace Elements Phase 3
31 Antidotes Phase 3
32 Micronutrients Phase 3
33 Liver Extracts Phase 3
34 Antiviral Agents Phase 3
35 Antimitotic Agents Phase 3
36 Tubulin Modulators Phase 3
37
Methylprednisolone Acetate Phase 3 584547
38 Antirheumatic Agents Phase 2, Phase 3
39 Folic Acid Antagonists Phase 2, Phase 3
40 Folate Phase 2, Phase 3
41 Vitamin B9 Phase 2, Phase 3
42 Vitamin B Complex Phase 2, Phase 3
43 Dermatologic Agents Phase 2, Phase 3
44 Anti-Inflammatory Agents, Non-Steroidal Phase 2, Phase 3
45 Analgesics, Non-Narcotic Phase 2, Phase 3
46 Analgesics Phase 2, Phase 3
47 Immunoglobulins, Intravenous Phase 2, Phase 3
48 Immunoglobulins Phase 2, Phase 3
49 2-chloro-3'-deoxyadenosine Phase 2, Phase 3 14485373
50 Cyclooxygenase Inhibitors Phase 2, Phase 3

Interventional clinical trials:

(show top 50) (show all 61)
# Name Status NCT ID Phase Drugs
1 A Trial to Evaluate the Long Term Prognosis in Rosai-Dorfman Disease Recruiting NCT05284942 Phase 4 Mycophenolate mofetil combined with oral prednisone
2 H-9926-LCH III: Treatment Protocol of the Third International Study for Langerhans Cell Histiocytosis Completed NCT00488605 Phase 3 Prednisone, Vinblastin, 6-mercaptopuroine;Leucovorin, Methotrexate, Vinblastine, Prednisone
3 Randomization of Cytarabine Monotherapy Versus Standard-of-Care Vinblastine/Prednisone for Frontline Treatment of Langerhans Cell Histiocytosis (TXCH LCH0115) Recruiting NCT02670707 Phase 3 Cytarabine;Vinblastine/prednisone
4 A Prospective Institutional Study for the Treatment of Children With Newly Diagnosed Langerhans Cell Histiocytosis Using a Cytarabine Contained Protocol Recruiting NCT04773366 Phase 3 Prednisone+Cytarabine+vincristine;Prednisone+Cytarabine+vincristine+Mercaptopurine;Prednisone+vincristine+Mercaptopurine;Prednisone+vincristine
5 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
6 In-vivo T-cell Depletion and Hematopoietic Stem Cell Transplantation for Life-Threatening Immune Deficiencies and Histiocytic Disorders Terminated NCT00176826 Phase 2, Phase 3 Myeloablative conditioning regimen
7 The Use Of Umbilical Cord Blood As A Source Of Hematopoietic Stem Cells Unknown status NCT00084695 Phase 2 busulfan;cyclophosphamide;fludarabine phosphate;melphalan;methylprednisolone
8 The Prospective Non-randomized Phase II Clinical Trial of Vemurafenib in Combination With Cytarabine and 2-chlorodeoxyadenosine in Children With Langerhans-cell Hisitocytosis With BRAF V600E Mutation Unknown status NCT03585686 Phase 2 Vemurafenib;Cytarabine;2-chlorodeoxyadenosine
9 Transplantation Using Umbilical Cord And Placental Blood Unknown status NCT00008164 Phase 2
10 Evaluation of Efficacy of Denosumab in Adult Patients With Langerhans Cell Histiocytosis (LCH): a Multiple-site, Single Arm, Open Label Clinical Trial Completed NCT03270020 Phase 2 Denosumab 70 MG/ML [Xgeva]
11 Efficacy and Tolerance of Combination Chemotherapy With Methotrexate and Cytosine Arabinoside in Newly Diagnosed Adult With Langerhans Cell Histiocytosis Completed NCT02389400 Phase 2 methotrexate
12 Treatment of Resistant Langerhans Cell Histiocytosis With Etanercept (ENBREL, IMMUNEX, SEATTLE) Completed NCT00048373 Phase 2 Etanercept
13 A Phase 2a, Open Label, Multicenter Study to Assess the Efficacy and Safety of the Oral AKT Inhibitor GSK2110183 in Subjects With Langerhans Cell Histiocytosis Completed NCT01395004 Phase 2 GSK2110183
14 Cord Blood Ex-Vivo MSC Expansion Plus Fucosylation to Enhance Homing and Engraftment Completed NCT03096782 Phase 2 Busulfan;Clofarabine;Cyclophosphamide;Fludarabine;Melphalan;Mycophenolate Mofetil;Tacrolimus
15 Allogeneic Hematopoietic Stem Cell Transplant for Patients With Immunologic or Histiocytic Disorders Using a Non-Myeloablative Preparative Regimen to Achieve Stable Mixed Chimerism Completed NCT00176865 Phase 2 Fludarabine;Melphalan;Anti-thymocyte globulin (ATG);Campath 1H;Cyclosporin A;Mycophenolate mofetil;Intravenous immunoglobulin (IVIG)
16 Study of Sequential Administration of Oral 6-Thioguanine After Methotrexate in Patients With Langerhans Cell in Histiocytosis (LCH) Completed NCT00588536 Phase 2 Methotrexate;6-Thioguanine;Leucovorin Calcium
17 Impact of Intervention With Deferasirox on the Immune Function of Patients With Hematologic Diseases and Transfusion-Related Iron Overload Completed NCT01273766 Phase 2 deferasirox
18 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) Screening Protocol Recruiting NCT03155620 Phase 2 Ensartinib;Erdafitinib;Larotrectinib Sulfate;Olaparib;Palbociclib;Samotolisib;Selpercatinib;Selumetinib Sulfate;Tazemetostat;Tipifarnib;Ulixertinib;Vemurafenib
19 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice)- Phase 2 Subprotocol of Tipifarnib in Patients With Tumors Harboring HRAS Genomic Alterations Recruiting NCT04284774 Phase 2 Tipifarnib
20 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of AG-120 (Ivosidenib) in Patients With Tumors Harboring IDH1 Mutations Recruiting NCT04195555 Phase 2 Ivosidenib
21 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of LOXO-292 in Patients With Tumors Harboring RET Gene Alterations Recruiting NCT04320888 Phase 2 Selpercatinib
22 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of LOXO-101 (Larotrectinib) in Patients With Tumors Harboring Actionable NTRK Fusions Recruiting NCT03213704 Phase 2 Larotrectinib Sulfate
23 NCI-COG Pediatric MATCH (Molecular Analysis For Therapy Choice)- Phase 2 Subprotocol of LY3023414 in Patients With Solid Tumors Recruiting NCT03213678 Phase 2 Samotolisib
24 Thalidomide, Cyclophosphamide and Dexamethasone for Adult Patients With Recurrent/Refractory Langerhans Cell Histiocytosis: A Single Arm, Single Center, Prospective Phase 2 Study Recruiting NCT04120519 Phase 2 thalidomide combined with dexamethasone and cyclophosphamide
25 Cytarabine Monotherapy for Adult Patients With Newly Diagnosed Langerhans Cell Histiocytosis: A Single Arm, Single Center, Prospective Phase 2 Study Recruiting NCT04121819 Phase 2 Cytarabine
26 A Phase 2 Study to Assess the Safety and Efficacy of Cobimetinib in Refractory Langerhans Cell Histiocytosis, LCH-Associated Neurodegenerative Disease, and Other Histiocytic Disorders. Recruiting NCT04079179 Phase 2 Cobimetinib
27 A Single-arm, Open Label, Multicenter Phase II Clinical Study in Rare Diseases to Evaluate Safety, Efficacy and PK of HLX208 for Adult Langerhans Cell Histiocytosis (LCH) and Erdheim-Chester Disease (ECD) With BRAF V600E Mutation Recruiting NCT05092815 Phase 2 HLX208
28 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of BVD-523FB (Ulixertinib) in Patients With Tumors Harboring Activating MAPK Pathway Mutations Active, not recruiting NCT03698994 Phase 2 Ulixertinib
29 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of Palbociclib in Patients With Tumors Harboring Activating Alterations in Cell Cycle Genes Active, not recruiting NCT03526250 Phase 2 Palbociclib
30 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice)- A Phase 2 Subprotocol of Olaparib in Patients With Tumors Harboring Defects in DNA Damage Repair Genes Active, not recruiting NCT03233204 Phase 2 Olaparib
31 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of Tazemetostat in Patients With Tumors Harboring Alterations in EZH2 or Members of the SWI/SNF Complex Active, not recruiting NCT03213665 Phase 2 Tazemetostat
32 Phase II Study of Clofarabine in Patients With Recurrent or Refractory Langerhans Cell Histiocytosis and LCH-related Disorders Active, not recruiting NCT02425904 Phase 2 Clofarabine
33 Evaluation of Efficacy and Tolerance of Cladribine in Symptomatic Patients With Pulmonary Langerhans Cell Histiocytosis and Impairment of Lung Function Active, not recruiting NCT01473797 Phase 2 Cladribine
34 CD34+ Stem Cell Selection for Patients Receiving a Matched or Partially Matched Family or Unrelated Adult Donor Allogeneic Stem Cell Transplantation for Non-Malignant Disease Active, not recruiting NCT01966367 Phase 1, Phase 2
35 A Phase II Study of Lenalidomide for Adult Histiocyte Disorders Active, not recruiting NCT02523040 Phase 2 Lenalidomide
36 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice)- Phase 2 Subprotocol of Vemurafenib in Patients With Tumors Harboring BRAF V600 Mutations Active, not recruiting NCT03220035 Phase 2 Vemurafenib
37 Phase 2 Study of DAY101 in Relapsed and Refractory Langerhans Cell Histiocytosis Not yet recruiting NCT05287295 Phase 2 Tovorafenib
38 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice)- Phase 2 Subprotocol of Ensartinib in Patients With Tumors Harboring ALK or ROS1 Genomic Alterations Suspended NCT03213652 Phase 2 Ensartinib
39 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of Erdafitinib in Patients With Tumors Harboring FGFR1/2/3/4 Alterations Suspended NCT03210714 Phase 2 Erdafitinib
40 Reduced Intensity Hematopoietic Cell Transplantation for Patients With Resistant Langerhans Cell Histiocytosis Terminated NCT00618540 Phase 2 fludarabine phosphate;melphalan
41 Phase II Study of Clofarabine in Patients With Recurrent or Refractory Langerhans Cell Histiocytosis Withdrawn NCT01796405 Phase 2 Clofarabine
42 A Phase 1/Pharmacokinetic Study of Sunitinib in Patients With Cancer Who Also Have HIV and Are on HAART Therapy Completed NCT00890747 Phase 1 sunitinib malate
43 Safety and Efficacy of CD207 Targeted CAR-T Cell Therapy in Patients With Relapsed and Refractory (R/R) Langerhans Cell Histiocytosis Not yet recruiting NCT05477446 Phase 1
44 A Phase I Study of [111In-DTPA-D-Phe]-Octreotide in Patients With Refractory Malignancies Expressing Somatostatin Receptors Terminated NCT00002947 Phase 1
45 Assessment of Safety of Air Travel in Patients With Pulmonary Langerhans Cell Histiocytosis Unknown status NCT03052101
46 CARDIOVASCULAR RISK FACTORS IN ADULT PATIENTS WITH MULTISYSTEM LANGERHANS-CELL HISTIOCYTOSIS: EVIDENCE OF GLUCOSE METABOLISM ABNORMALITIES Unknown status NCT00483925
47 Evaluation of Exercise Capacity and Mechanisms of Exercise Limitation in Patients With Pulmonary Langerhans Cell Histiocytosis Completed NCT02665546
48 Evaluation of Outcome of Adult Pulmonary Langerhans Cell Histiocytosis Based on Lung HRCT and Lung Function. A Multicenter Retrospective Study Completed NCT01651507
49 Treatment Protocol of the Third International Study For Langerhans Cell Histiocytosis Completed NCT00276757 leucovorin calcium;methotrexate;prednisolone;vinblastine sulfate
50 Epidemiology of Adult Adult Pulmonary Langerhans Cell Histiocytosis - A Multicenter Cohort Study Completed NCT01225601

Search NIH Clinical Center for Langerhans Cell Histiocytosis

Inferred drug relations via UMLS 71 / NDF-RT 50 :


Thalidomide

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 28

Anatomical Context for Langerhans Cell Histiocytosis

Organs/tissues related to Langerhans Cell Histiocytosis:

MalaCards : Spleen, Bone Marrow, Bone, Pituitary, Thyroid, Lung, Skin

Publications for Langerhans Cell Histiocytosis

Articles related to Langerhans Cell Histiocytosis:

(show top 50) (show all 6626)
# Title Authors PMID Year
1
Interleukin-17A is not expressed by CD207(+) cells in Langerhans cell histiocytosis lesions. 62 57
19424201 2009
2
Langerhans cell histiocytosis reveals a new IL-17A-dependent pathway of dendritic cell fusion. 62 57
18157139 2008
3
Hypothalamo-pituitary abnormalities in adult patients with langerhans cell histiocytosis: clinical, endocrinological, and radiological features and response to treatment. 62 57
10770168 2000
4
Familial clustering of Langerhans cell histiocytosis. 62 57
10606898 1999
5
Langerhans cell histiocytosis. 62 57
7608790 1995
6
Differential immunophenotypic analysis of dendritic cell tumours. 53 62
20439325 2010
7
Immunocytochemical investigation of Langerin (CD207) is a valuable adjunct in the cytological diagnosis of Langerhans cell histiocytosis of the thyroid. 53 62
19195798 2009
8
Immunohistochemical expression of Langerin in Langerhans cell histiocytosis and non-Langerhans cell histiocytic disorders. 53 62
18277880 2008
9
In vivo transformation of mouse conventional CD8alpha+ dendritic cells leads to progressive multisystem histiocytosis. 53 62
18029555 2008
10
Langerhans' cell histiocytosis with multisystem involvement in an adult. 53 62
17953644 2007
11
Immunohistochemical analysis of langerin in langerhans cell histiocytosis and pulmonary inflammatory and infectious diseases. 53 62
17527085 2007
12
[A case of pulmonary langerhans cell histiocytosis presenting disappearance of coalescing air wall cysts after smoking cessation]. 53 62
16050471 2005
13
Tumor necrosis factor, interleukin 11, and leukemia inhibitory factor produced by Langerhans cells in Langerhans cell histiocytosis. 53 62
15543003 2004
14
Diagnostic relevance of Langerin detection in cells from bronchoalveolar lavage of patients with pulmonary Langerhans cell histiocytosis, sarcoidosis and idiopathic pulmonary fibrosis. 53 62
14722767 2004
15
Atypical generalized eruptive histiocytosis associated with acute monocytic leukemia. 53 62
14576638 2003
16
Langerhans cell histiocytosis immunohistochemical expression of fascin, a dendritic cell marker. 53 62
12219775 2002
17
Abundant expression of CD40 and CD40-ligand (CD154) in paediatric Langerhans cell histiocytosis lesions. 53 62
11044648 2000
18
DNA polymorphisms and mutations of the tumor necrosis factor-alpha (TNF-alpha) promoter in Langerhans cell histiocytosis (LCH). 53 62
9355965 1997
19
Molecular analysis of the t(15;17) translocation in de novo and secondary acute promyelocytic leukemia. 53 62
9209367 1997
20
Nonlipidized juvenile xanthogranuloma: a histologic and immunohistochemical study. 53 62
9144693 1997
21
Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases. 53 62
8965684 1996
22
Modulation of fibroblast activity in histiocytosis X by platelet-derived growth factor. 53 62
7874940 1995
23
Immunohistochemical demonstration of the lysosome-associated glycoprotein CD68 (KP-1) in granular cell tumors and schwannomas. 53 62
7959661 1994
24
Cathepsin D and E co-expression in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and Langerhans' cell histiocytosis: further evidences of a phenotypic overlap between these histiocytic disorders. 53 62
8055153 1994
25
Immunohistochemical detection of granulocyte/macrophage colony-stimulating factor in Langerhans' cell histiocytosis. 53 62
7507881 1993
26
Histologic and immunohistochemical study comparing xanthoma disseminatum and histiocytosis X. 53 62
1519935 1992
27
Histiocytosis X. S-100 protein, peanut agglutinin, and transmission electron microscopy study. 53 62
2239828 1990
28
Histiocytosis X of the vulva: a case report and review of the literature. 53 62
2406669 1990
29
A case of renal involvement in juvenile xanthogranulomatosis. 62
36439921 2023
30
Outcomes of liver transplantation in children with Langerhans cell histiocytosis: Experience from a quaternary care center. 62
36317422 2023
31
PET/CT Showing a Case of Langerhans Cell Histiocytosis Involving the Pleura. 62
36469071 2023
32
The added value of skeletal surveys in the initial evaluation of children diagnosed with Langerhans cell histiocytosis in the era of staging 18 F-FDG PET/CT: A retrospective study. 62
36266951 2023
33
Histiocytic Diseases. 62
36270835 2023
34
Langerhans Cell Histiocytosis Appearing as Wandering Pulmonary Nodules. 62
35342142 2022
35
Classic cytomorphological features of Langerhans cell histiocytosis with osseous involvement. 62
36047490 2022
36
Diagnosis of Langerhans cell histiocytosis on cytological examination of cerebrospinal fluid: Report of the first case. 62
35950687 2022
37
Optimal timing of liver transplantation for liver cirrhosis caused by sclerosing cholangitis in a patient with Langerhans cell histiocytosis: a case report. 62
36469185 2022
38
Preceding polydipsia/polyuria, ataxia, and dysarthria in an adult with mixed histiocytosis (Erdheim Chester disease/Langerhans cell histiocytosis). 62
36197468 2022
39
Kingella kingae Osteomyelitis Should be Ruled out in Pediatric Langerhans Cell Histiocytosis. 62
36102720 2022
40
Incidence, prevalence and survival in patients with Langerhans cell histiocytosis: A national registry study from England, 2013-2019. 62
36122574 2022
41
High-Resolution Computed Tomography of Cystic Lung Disease. 62
36252611 2022
42
Narrowing the Differential Diagnosis of Cystic Lesions in Smokers with Expiratory CT Acquisition Using the Cyst-Airway Communication Hypothesis. 62
36271930 2022
43
Biomodulatory therapy induces durable remissions in multi-system Langerhans cell histiocytosis. 62
35819881 2022
44
The international dataset on the association between Langerhans Cell Histiocytosis and other malignancies. 62
36188132 2022
45
A Review of Noninfectious Diseases Masquerading as Acute Mastoiditis. 62
34874762 2022
46
Strong Coexpression of Transcription Factors PU.1 and Oct-2 in Rosai-Dorfman Disease. 62
36239684 2022
47
68Ga-FAPI and 18F-FDG PET/CT Images of a Patient With Rosai-Dorfman Disease With Liver Involvement. 62
36342795 2022
48
Paediatric Strategy Forum for medicinal product development in mitogen-activated protein kinase pathway inhibitors: ACCELERATE in collaboration with the European Medicines Agency with participation of the Food and Drug Administration. 62
36335782 2022
49
Curative Pericardiectomy in Interpheron-Resistant Severe Pericardial Erdheim-Chester Disease. 62
36444187 2022
50
Congenital solitary reticulohistiocytosis (Hashimoto - Pritzker). 62
36153176 2022

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 GeneCards Suite gene sharing:

(show all 25)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.53 TNF NRAS MAP2K1 LIF IL17A IL11
2
Show member pathways
13.13 TNF SERPINA3 S100B NRAS MAP2K1 LIF
3
Show member pathways
12.93 CSF2 F13A1 FSCN1 IL11 IL17A LIF
4
Show member pathways
12.76 NRAS MAP2K1 LIF IL11 BRAF
5
Show member pathways
12.34 NRAS MAP2K1 LIF BRAF
6 12.27 TNF NRAS MAP2K1 BRAF
7
Show member pathways
12.14 TNF MAP2K1 IL17A CSF2
8
Show member pathways
11.99 TNF MAP2K1 IL17A CSF2
9 11.78 TNF IL11 CSF2 CD68 CD1C
10 11.69 TNF LIF IL17A FSCN1 F13A1
11 11.68 TNF IL17A CD101
12
Show member pathways
11.67 NRAS MAP2K1 BRAF
13
Show member pathways
11.6 IL11 LIF SERPINA3
14
Show member pathways
11.53 NRAS MAP2K1 BRAF
15
Show member pathways
11.51 NRAS MAP2K1 BRAF
16 11.38 TNF LIF CSF2
17 11.33 NRAS MAP2K1 BRAF
18 11.27 NRAS MAP2K1 BRAF
19 11.23 BRAF MAP2K1 NRAS
20 11.22 TNF CSF2 CD207 CD1C CD1A
21 11.09 TNF NRAS MAP2K1
22 11.04 TNF IL11 CSF2
23 10.98 TNF IL17A CSF2
24 10.84 TNF MAP2K1 LIF IL17A IL11 CSF2
25 10.65 MAP2K1 BRAF

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 external side of plasma membrane GO:0009897 9.8 TNF IL17A CD207 CD1E CD1C CD1B
2 extracellular space GO:0005615 9.7 TNF SERPINA3 S100B LIF IL17A IL11

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

# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 10.07 CD1A CD1B CD1C CD1E CSF2 IL17A
2 MAPK cascade GO:0000165 10.01 TNF NRAS MAP2K1 BRAF
3 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.97 TNF LIF IL11 BRAF
4 positive regulation of podosome assembly GO:0071803 9.8 TNF FSCN1 CSF2
5 positive regulation of interleukin-23 production GO:0032747 9.76 IL17A CSF2
6 positive regulation of T cell mediated cytotoxicity GO:0001916 9.76 CD1E CD1C CD1B CD1A
7 regulation of axon regeneration GO:0048679 9.61 MAP2K1 BRAF
8 antigen processing and presentation, exogenous lipid antigen via MHC class Ib GO:0048007 9.56 CD1E CD1C CD1B CD1A
9 antigen processing and presentation, endogenous lipid antigen via MHC class Ib GO:0048006 9.23 CD1E CD1C CD1B CD1A

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 9.96 TNF LIF IL17A IL11 CSF2
2 lipopeptide binding GO:0071723 9.76 CD1E CD1C CD1B CD1A
3 exogenous lipid antigen binding GO:0030884 9.56 CD1E CD1C CD1B CD1A
4 endogenous lipid antigen binding GO:0030883 9.23 CD1E CD1C CD1B CD1A

Sources for Langerhans Cell Histiocytosis

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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