MCID: ADL082
MIFTS: 21

Adult Pulmonary Langerhans Cell Histiocytosis malady

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

Aliases & Classifications for Adult Pulmonary Langerhans Cell Histiocytosis

Aliases & Descriptions for Adult Pulmonary Langerhans Cell Histiocytosis:

Name: Adult Pulmonary Langerhans Cell Histiocytosis 56 69
Pulmonary Histiocytosis X 56 69

Characteristics:

Orphanet epidemiological data:

56
adult pulmonary langerhans cell histiocytosis
Inheritance: Not applicable; Age of onset: Adult;

Classifications:



External Ids:

Orphanet 56 ORPHA99874
ICD10 via Orphanet 34 C96.5

Summaries for Adult Pulmonary Langerhans Cell Histiocytosis

MalaCards based summary : Adult Pulmonary Langerhans Cell Histiocytosis, also known as pulmonary histiocytosis x, is related to histiocytosis and pulmonary hypertension, and has symptoms including tapered finger, dyspnea and abnormal pattern of respiration. The drugs Cyclophosphamide and Busulfan have been mentioned in the context of this disorder. Affiliated tissues include bone and lung.

Related Diseases for Adult Pulmonary Langerhans Cell Histiocytosis

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

id Related Disease Score Top Affiliating Genes
1 histiocytosis 10.2
2 pulmonary hypertension 9.8

Symptoms & Phenotypes for Adult Pulmonary Langerhans Cell Histiocytosis

Human phenotypes related to Adult Pulmonary Langerhans Cell Histiocytosis:

56 32
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 tapered finger 56 32 Occasional (29-5%) HP:0001182
2 dyspnea 56 32 Very frequent (99-80%) HP:0002094
3 abnormal pattern of respiration 56 32 Very frequent (99-80%) HP:0002793
4 cough 56 32 Very frequent (99-80%) HP:0012735
5 chest pain 56 32 Occasional (29-5%) HP:0100749

Drugs & Therapeutics for Adult Pulmonary Langerhans Cell Histiocytosis

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

(show top 50) (show all 101)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
2
Busulfan Approved, Investigational Phase 2, Phase 3 55-98-1 2478
3
Prednisolone Approved, Vet_approved Phase 2, Phase 3 50-24-8 5755
4
Methylprednisolone Approved, Vet_approved Phase 2, Phase 3 83-43-2 6741
5
Vinblastine Approved Phase 3,Phase 2 865-21-4 13342 241903
6
Methotrexate Approved Phase 3,Phase 2 1959-05-2, 59-05-2 126941
7
Prednisone Approved, Vet_approved Phase 3,Phase 2 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 3 147-94-4 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 58-05-9 54575, 6560146 143
13
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3,Phase 2 59-30-3 6037
14 Prednisolone acetate Phase 2, Phase 3
15 Alkylating Agents Phase 2, Phase 3
16 glucocorticoids Phase 3,Phase 2
17 Hormone Antagonists Phase 3,Phase 2,Phase 1
18 Hormones Phase 3,Phase 2,Phase 1
19 Peripheral Nervous System Agents Phase 2, Phase 3
20 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3,Phase 2,Phase 1
21 Prednisolone hemisuccinate Phase 2, Phase 3
22 Prednisolone phosphate Phase 2, Phase 3
23 Immunosuppressive Agents Phase 2, Phase 3
24 Anti-Inflammatory Agents Phase 3,Phase 2
25 Antimetabolites Phase 3,Phase 2
26 Antimetabolites, Antineoplastic Phase 3,Phase 2
27 Methylprednisolone acetate Phase 2, Phase 3
28 Methylprednisolone Hemisuccinate Phase 2, Phase 3
29 Antirheumatic Agents Phase 3,Phase 2
30 Antineoplastic Agents, Alkylating Phase 2, Phase 3
31 Antineoplastic Agents, Hormonal Phase 3,Phase 2
32 Analgesics Phase 2, Phase 3
33 Folic Acid Antagonists Phase 3,Phase 2
34 Nucleic Acid Synthesis Inhibitors Phase 3,Phase 2
35 Analgesics, Non-Narcotic Phase 2, Phase 3
36 Dermatologic Agents Phase 3,Phase 2
37 Vitamin B Complex Phase 3,Phase 2
38 Immunoglobulins Phase 2, Phase 3
39 gamma-Globulins Phase 2, Phase 3
40 6-Mercaptopurine Phase 3,Phase 2
41 Anti-Infective Agents Phase 2, Phase 3
42 Immunoglobulins, Intravenous Phase 2, Phase 3
43 Anti-Inflammatory Agents, Non-Steroidal Phase 2, Phase 3
44 Rho(D) Immune Globulin Phase 2, Phase 3
45 Antibodies Phase 2, Phase 3
46 Antimitotic Agents Phase 3,Phase 2
47 Antineoplastic Agents, Phytogenic Phase 3,Phase 2
48 Cyclooxygenase Inhibitors Phase 2, Phase 3
49 Tocolytic Agents Phase 2, Phase 3
50 2-chloro-3'-deoxyadenosine Phase 2, Phase 3

Interventional clinical trials:

(show all 30)
id Name Status NCT ID Phase
1 T-Cell Depletion and Stem Cell Transplant for Immune Deficiencies and Histiocytic Disorders Unknown status NCT00176826 Phase 2, Phase 3
2 H-9926-LCH III: Treatment Protocol of the Third International Study for Langerhans Cell Histiocytosis Completed NCT00488605 Phase 3
3 LCH-IV, International Collaborative Treatment Protocol for Children and Adolescents With Langerhans Cell Histiocytosis Recruiting NCT02205762 Phase 2, Phase 3
4 Vinblastine/Prednisone Versus Single Therapy With Cytarabine for Langerhans Cell Histiocytosis (LCH) Recruiting NCT02670707 Phase 3
5 A Study to Test the Ability of and Safety of GSK2110183 in Treating Langerhans Cell Histiocytosis Unknown status NCT01395004 Phase 2
6 Umbilical Cord Blood and Placental Blood Transplantation in Treating Patients With Hematologic Cancer or Aplastic Anemia Unknown status NCT00008164 Phase 2
7 Umbilical Cord Blood for Stem Cell Transplantation in Treating Young Patients With Malignant or Nonmalignant Diseases Unknown status NCT00084695 Phase 2
8 Treatment of Resistant Langerhans Cell Histiocytosis With ENBREL Completed NCT00048373 Phase 2
9 Study of Sequential Administration of Oral 6-Thioguanine After Methotrexate in Patients With LCH Completed NCT00588536 Phase 2
10 Stem Cell Transplant for Immunologic or Histiocytic Disorders Completed NCT00176865 Phase 2
11 Methotrexate and Cytosine in Adult Langerhans Cell Histiocytosis Recruiting NCT02389400 Phase 2
12 A Study of Lenalidomide for Adult Histiocyte Disorders Recruiting NCT02523040 Phase 2
13 Phase II Study of Clofarabine in Patients With Recurrent or Refractory Langerhans Cell Histiocytosis Recruiting NCT02425904 Phase 2
14 Evaluation of Efficacy and Tolerance of Cladribine in Symptomatic Pulmonary Langerhans Cell Histiocytosis Active, not recruiting NCT01473797 Phase 2
15 Pediatric MATCH: Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients With Advanced Refractory Solid Tumors or Lymphomas Not yet recruiting NCT03155620 Phase 2
16 Reduced Intensity Hematopoietic Cell Transplantation for Patients With Resistant Langerhans Cell Histiocytosis Terminated NCT00618540 Phase 2
17 Blood Stem Cell Transplant in Treating Patients With Hematologic Cancer Terminated NCT00008216 Phase 2
18 Clofarabine for Langerhans in Pedi Withdrawn NCT01796405 Phase 2
19 Sunitinib Malate in Treating HIV-Positive Patients With Cancer Receiving Antiretroviral Therapy Completed NCT00890747 Phase 1
20 Indium In 111 Pentetreotide in Treating Patients With Refractory Cancer Terminated NCT00002947 Phase 1
21 Cardiovascular Risk Factors and LCH in Adults Unknown status NCT00483925
22 Prospective Evaluation of Adult Pulmonary Langerhans Cell Histiocytosis Completed NCT01225601
23 Evaluation of Outcome of Adult Pulmonary Langerhans Cell Histiocytosis Based on Lung HRCT and Lung Function Completed NCT01651507
24 Deferasirox in Treating Iron Overload Caused By Blood Transfusions in Patients With Hematologic Malignancies Completed NCT01273766
25 Assessment of Safety of Air Travel in Patients With Pulmonary Langerhans Cell Histiocytosis Recruiting NCT03052101
26 Evaluation of Exercise Capacity and Exercise Limitation in Patients With Pulmonary Langerhans Cell Histiocytosis Recruiting NCT02665546
27 Characterization of Pulmonary Langerhans Cell Histiocytosis Recruiting NCT03093727
28 CD34+ Stem Cell Selection for Patients Receiving Allogeneic Stem Cell Transplantation for Non-Malignant Disease Recruiting NCT01966367
29 Uptake and Biodistribution of 18F-fluorocholine in Histiocytic Disorders by PET Imaging and Biopsy Measurement Recruiting NCT02608619
30 Allogeneic Hematopoietic Stem Cell Transplant for Patients With Primary Immune Deficiencies Recruiting NCT01652092

Search NIH Clinical Center for Adult Pulmonary Langerhans Cell Histiocytosis

Genetic Tests for Adult Pulmonary Langerhans Cell Histiocytosis

Anatomical Context for Adult Pulmonary Langerhans Cell Histiocytosis

MalaCards organs/tissues related to Adult Pulmonary Langerhans Cell Histiocytosis:

39
Bone, Lung

Publications for Adult Pulmonary Langerhans Cell Histiocytosis

Articles related to Adult Pulmonary Langerhans Cell Histiocytosis:

id Title Authors Year
1
The natural history of adult pulmonary Langerhans cell histiocytosis: a prospective multicentre study. ( 25887097 )
2015
2
Severe pulmonary hypertension in adult pulmonary Langerhans cell histiocytosis: the effect of sildenafil as a bridge to lung transplantation. ( 25175135 )
2014
3
A prospective clinical multicentre study on adult pulmonary Langerhans' cell histiocytosis. ( 23461076 )
2012
4
Effect of pregnancy on lung function in adult pulmonary Langerhans cell histiocytosis. ( 16492595 )
2006
5
Evidence that Langerhans cells in adult pulmonary Langerhans cell histiocytosis are mature dendritic cells: importance of the cytokine microenvironment. ( 10477625 )
1999
6
Relapsing nodular lesions in the course of adult pulmonary Langerhans cell histiocytosis. ( 9620939 )
1998

Variations for Adult Pulmonary Langerhans Cell Histiocytosis

Expression for Adult Pulmonary Langerhans Cell Histiocytosis

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

Pathways for Adult Pulmonary Langerhans Cell Histiocytosis

GO Terms for Adult Pulmonary Langerhans Cell Histiocytosis

Sources for Adult Pulmonary Langerhans Cell Histiocytosis

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