MCID: ADL082
MIFTS: 21

Adult Pulmonary Langerhans Cell Histiocytosis

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

Aliases & Classifications for Adult Pulmonary Langerhans Cell Histiocytosis

MalaCards integrated aliases 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
UMLS via Orphanet 70 C1455705
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 dyspnea, cough and chest pain. The drugs Cytarabine and Methotrexate have been mentioned in the context of this disorder. Affiliated tissues include lung and bone.

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
3 germ cells tumors 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 dyspnea 56 32 hallmark (90%) Very frequent (99-80%) HP:0002094
2 cough 56 32 hallmark (90%) Very frequent (99-80%) HP:0012735
3 chest pain 56 32 occasional (7.5%) Occasional (29-5%) HP:0100749
4 tapered finger 56 32 occasional (7.5%) Occasional (29-5%) HP:0001182
5 abnormal pattern of respiration 56 32 hallmark (90%) Very frequent (99-80%) HP:0002793

Drugs & Therapeutics for Adult Pulmonary Langerhans Cell Histiocytosis

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

(show all 17)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cytarabine Approved, Investigational Phase 2 147-94-4 6253
2
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 126941
3
Cladribine Approved, Investigational Phase 2 4291-63-8 20279
4
leucovorin Approved, Nutraceutical Phase 2 58-05-9 143 6006
5
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
6 Folic Acid Antagonists Phase 2
7 Dermatologic Agents Phase 2
8 Nucleic Acid Synthesis Inhibitors Phase 2
9 Vitamin B Complex Phase 2
10 Anti-Infective Agents Phase 2
11 Immunosuppressive Agents Phase 2
12 Antimetabolites Phase 2
13 Antimetabolites, Antineoplastic Phase 2
14 Antirheumatic Agents Phase 2
15 Antiviral Agents Phase 2
16 Folate Nutraceutical Phase 2
17 Vitamin B9 Nutraceutical Phase 2

Interventional clinical trials:


id Name Status NCT ID Phase Drugs
1 Methotrexate and Cytosine in Adult Langerhans Cell Histiocytosis Recruiting NCT02389400 Phase 2 methotrexate
2 Evaluation of Efficacy and Tolerance of Cladribine in Symptomatic Pulmonary Langerhans Cell Histiocytosis Active, not recruiting NCT01473797 Phase 2 Cladribine
3 Prospective Evaluation of Adult Pulmonary Langerhans Cell Histiocytosis Completed NCT01225601
4 Evaluation of Outcome of Adult Pulmonary Langerhans Cell Histiocytosis Based on Lung HRCT and Lung Function Completed NCT01651507
5 Assessment of Safety of Air Travel in Patients With Pulmonary Langerhans Cell Histiocytosis Recruiting NCT03052101
6 Evaluation of Exercise Capacity and Exercise Limitation in Patients With Pulmonary Langerhans Cell Histiocytosis Recruiting NCT02665546
7 Characterization of Pulmonary Langerhans Cell Histiocytosis Recruiting NCT03093727

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
Lung, Bone

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 HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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