MCID: ADL082
MIFTS: 22

Adult Pulmonary Langerhans Cell Histiocytosis

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

Aliases & Classifications for Adult Pulmonary Langerhans Cell Histiocytosis

MalaCards integrated aliases for Adult Pulmonary Langerhans Cell Histiocytosis:

Name: Adult Pulmonary Langerhans Cell Histiocytosis 60 74
Pulmonary Histiocytosis X 60 74

Characteristics:

Orphanet epidemiological data:

60
adult pulmonary langerhans cell histiocytosis
Inheritance: Not applicable; Age of onset: Adult;

Classifications:



External Ids:

ICD10 via Orphanet 35 C96.5
UMLS via Orphanet 75 C1455705
Orphanet 60 ORPHA99874

Summaries for Adult Pulmonary Langerhans Cell Histiocytosis

MalaCards based summary : Adult Pulmonary Langerhans Cell Histiocytosis, also known as pulmonary histiocytosis x, is related to langerhans cell histiocytosis and histiocytosis. Affiliated tissues include lung, bone and lymph node, and related phenotypes are dyspnea and cough

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:

(show all 11)
# Related Disease Score Top Affiliating Genes
1 langerhans cell histiocytosis 10.5
2 histiocytosis 10.5
3 pulmonary hypertension 10.0
4 endocarditis 10.0
5 bronchus cancer 9.9
6 pneumothorax 9.9
7 interstitial lung disease 9.9
8 lung disease 9.9
9 hodgkin's lymphoma, lymphocytic-histiocytic predominance 9.9
10 diabetes insipidus 9.9
11 eosinophilic granuloma 9.9

Graphical network of the top 20 diseases related to Adult Pulmonary Langerhans Cell Histiocytosis:



Diseases related to Adult Pulmonary Langerhans Cell Histiocytosis

Symptoms & Phenotypes for Adult Pulmonary Langerhans Cell Histiocytosis

Human phenotypes related to Adult Pulmonary Langerhans Cell Histiocytosis:

60 33
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 dyspnea 60 33 hallmark (90%) Very frequent (99-80%) HP:0002094
2 cough 60 33 hallmark (90%) Very frequent (99-80%) HP:0012735
3 abnormal pattern of respiration 60 33 hallmark (90%) Very frequent (99-80%) HP:0002793
4 chest pain 60 33 occasional (7.5%) Occasional (29-5%) HP:0100749
5 tapered finger 60 33 occasional (7.5%) Occasional (29-5%) HP:0001182

Drugs & Therapeutics for Adult Pulmonary Langerhans Cell Histiocytosis

Search Clinical Trials , 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:

42
Lung, Bone, Lymph Node

Publications for Adult Pulmonary Langerhans Cell Histiocytosis

Articles related to Adult Pulmonary Langerhans Cell Histiocytosis:

(show top 50) (show all 71)
# 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
[Adult pulmonary Langerhans cell histiocytosis. Report of one case]. ( 21773662 )
2011
5
Effect of pregnancy on lung function in adult pulmonary Langerhans cell histiocytosis. ( 16492595 )
2006
6
Adult pulmonary Langerhans' cell histiocytosis. ( 16772390 )
2006
7
Pulmonary histiocytosis X in a patient with epithelioid mesothelioma. ( 16918985 )
2006
8
Clinico-epidemiological features of pulmonary histiocytosis X. ( 11688843 )
2001
9
Adult pulmonary Langerhans' cell histiocytosis. ( 10770823 )
2000
10
Streptococcus equinus endocarditis in a patient with pulmonary histiocytosis X. ( 10866592 )
2000
11
Evidence that Langerhans cells in adult pulmonary Langerhans cell histiocytosis are mature dendritic cells: importance of the cytokine microenvironment. ( 10477625 )
1999
12
Pulmonary histiocytosis X. ( 11155889 )
1999
13
Streptococcus equinus endocarditis in a patient with pulmonary histiocytosis X. ( 10680994 )
1999
14
Relapsing nodular lesions in the course of adult pulmonary Langerhans cell histiocytosis. ( 9620939 )
1998
15
Pulmonary histiocytosis X. ( 17344722 )
1998
16
Recurrent spontaneous pneumothorax in pulmonary histiocytosis X. ( 9798765 )
1998
17
Pulmonary vascular involvement in pulmonary histiocytosis X. ( 9404788 )
1997
18
Advanced pulmonary histiocytosis X is associated with severe pulmonary hypertension. ( 9106608 )
1997
19
Group II phospholipase A2 and pulmonary histiocytosis X. ( 9266909 )
1997
20
Epithelioid haemangioendothelioma of the lung imitating clinical features of pulmonary histiocytosis X. ( 9401364 )
1997
21
Pulmonary histiocytosis X: pulmonary function and exercise pathophysiology. ( 8542154 )
1996
22
Role of granulocyte-macrophage colony stimulating factor (GM-CSF) in the pathogenesis of adult pulmonary histiocytosis X. ( 8693443 )
1996
23
Alveolar macrophages from bronchoalveolar lavage of patients with pulmonary histiocytosis X: determination of phenotypic and functional changes. ( 7616760 )
1995
24
Pulmonary histiocytosis X diagnosed by bone biopsy. Report of a case. ( 8067180 )
1994
25
Clinical and radiologic features, lung function and therapeutic results in pulmonary histiocytosis X. ( 8469818 )
1993
26
Surface phenotype of Langerhans cells and lymphocytes in granulomatous lesions from patients with pulmonary histiocytosis X. ( 8503566 )
1993
27
Bronchogenic carcinoma in patients with pulmonary histiocytosis X. ( 1600781 )
1992
28
Imaging of pulmonary histiocytosis X. ( 1609142 )
1992
29
Value of CD-1-positive cells in bronchoalveolar lavage fluid for the diagnosis of pulmonary histiocytosis X. ( 1758200 )
1991
30
Increased expression of growth factor genes for macrophages and fibroblasts in bronchoalveolar lavage cells of a patient with pulmonary histiocytosis X. ( 1771606 )
1991
31
Pulmonary histiocytosis X cells phagocytize Staphylococcus aureus in vitro. ( 1783048 )
1991
32
Development of pulmonary histiocytosis X after chemotherapy for Hodgkin disease. ( 1698017 )
1990
33
Pulmonary histiocytosis X with mediastinal lymph node involvement. ( 2240846 )
1990
34
The clinical role of BAL in pulmonary histiocytosis X. ( 2292298 )
1990
35
Case of the day. Pediatric. Pulmonary histiocytosis X. ( 2343170 )
1990
36
Complete resolution of roentgenographic changes in a patient with pulmonary histiocytosis X. ( 2394160 )
1990
37
Pulmonary histiocytosis X: comparison of radiographic and CT findings. ( 2787035 )
1989
38
Pulmonary histiocytosis X: evaluation with high-resolution CT. ( 2787036 )
1989
39
Pulmonary histiocytosis X and carcinoma. ( 3493752 )
1987
40
Smoking and interstitial lung disease. The effect of cigarette smoking on the incidence of pulmonary histiocytosis X and sarcoidosis. ( 3488004 )
1986
41
A case report and literature review of "primary" pulmonary histiocytosis X of childhood. ( 3512972 )
1986
42
Pulmonary histiocytosis X. Immunoperoxidase staining for HLA-DR antigen and S100 protein. ( 3533003 )
1986
43
Immunocytochemical characterization of pulmonary histiocytosis X cells in lung biopsies. ( 3872076 )
1985
44
Pulmonary histiocytosis X. ( 3930893 )
1985
45
Diagnosis of pulmonary histiocytosis X by immunodetection of Langerhans cells in bronchoalveolar lavage fluid. ( 6372496 )
1984
46
Severe airways obstruction as a presenting feature of pulmonary histiocytosis-X: a case report. ( 6604536 )
1983
47
Langerhans cells in bronchoalveolar lavage in the late stages of pulmonary histiocytosis X. ( 6976256 )
1982
48
Chest radiological features of pulmonary histiocytosis X: a report based on 50 adult cases. ( 6979115 )
1982
49
Primary pulmonary histiocytosis X in two patients with Hodgkin's disease. ( 6979116 )
1982
50
Primary pulmonary histiocytosis-X. ( 6982147 )
1982

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

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35 ICD10 via Orphanet
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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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