PML
MCID: PRG009
MIFTS: 57

Progressive Multifocal Leukoencephalopathy (PML) malady

Infectious diseases, Neuronal diseases categories

Summaries for Progressive Multifocal Leukoencephalopathy

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Sources:
8Disease Ontology, 42NIH Rare Diseases, 43NINDS, 63Wikipedia, 32MalaCards
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NIH Rare Diseases:42 Progressive multifocal leukoencephalopathy (pml) is a neurological disorder that damages the myelin that covers and protects nerves in the white matter of the brain. it is caused by the jc virus (jcv). by age 10, most people have been infected with this virus, but it rarely causes symptoms unless the immune system becomes severely weakened. the disease occurs, rarely, in organ transplant patients; people undergoing chronic corticosteroid or immunosuppressive therapy; and individuals with cancer, such as hodgkin’s disease, lymphoma, and sarcoidosis.  pml is most common among individuals with acquired immune deficiency syndrome (aids). last updated: 4/1/2011

MalaCards: Progressive Multifocal Leukoencephalopathy, also known as leukoencephalopathy, progressive multifocal, is related to multiple sclerosis and leukemia. An important gene associated with Progressive Multifocal Leukoencephalopathy is IGHMBP2 (immunoglobulin mu binding protein 2), and among its related pathways is purine nucleotides degradation II (aerobic). The compounds deoxyinosine and 9-(beta-d-arabinofuranosyl)guanine have been mentioned in the context of this disorder. Affiliated tissues include brain, t cells and kidney, and related mouse phenotypes are skeleton and respiratory system.

Disease Ontology:8 A viral infectious disease that involves reactivation of jc polyomavirus in immune-compromised individuals which causes the loss of white matter (which is made up of myelin, a substance the surrounds and protects nerve fibers) in multiple areas of the brain. the symptoms include hemiparesis, aphasia, dysarthria, hemianopia, cognitive impairment and coma.

NINDS:43 Progressive multifocal leukoencephalopathy (PML) is a disease of the white matter of the brain, caused by a virus infection that targets cells that make myelin--the material that insulates nerve cells (neurons).

Wikipedia:63 Progressive multifocal leukoencephalopathy (PML), also known as progressive multifocal... more...

Aliases & Classifications for Progressive Multifocal Leukoencephalopathy

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8Disease Ontology, 42NIH Rare Diseases, 43NINDS, 10DISEASES, 48Orphanet, 60UMLS, 44Novoseek, 34MeSH, 27ICD9CM, 39NCIt, 56SNOMED-CT, 61UMLS via Orphanet, 35MESH via Orphanet, 57SNOMED-CT via Orphanet, 25ICD10
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Infectious diseases
Anatomical: Neuronal diseases


Characteristics (Orphanet epidemiological data):

48
progressive multifocal leukoencephalopathy:
Inheritance: Sporadic; Age of onset: Variable


Aliases & Descriptions:

progressive multifocal leukoencephalopathy 8 42 43 10 48
leukoencephalopathy, progressive multifocal 42 60
leukoencephalopathy progressive multifocal 44
progressive multifocal leukoencephalitis 48
pml 42


External Ids:

Disease Ontology8 DOID:643
MeSH34 D007968
ICD9CM27 046.3
NCIt39 C26815
SNOMED-CT56 22255007
UMLS via Orphanet61 C0023524
MESH via Orphanet35 D007968
SNOMED-CT via Orphanet57 22255007
ICD1025 A81.2

Related Diseases for Progressive Multifocal Leukoencephalopathy

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17GeneCards, 18GeneDecks
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Diseases related to Progressive Multifocal Leukoencephalopathy via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 175)
idRelated DiseaseScoreTop Affiliating Genes
1multiple sclerosis31.2PDCD1, ITGA4, LBR
2leukemia31.0PNP, ITGA4, CD9, SPIB, LBR, MECP2
3systemic lupus erythematosus30.9LBR, ADA, PDCD1, ITGA4
4acquired immunodeficiency syndrome30.8CARTPT, TAT
5arthritis30.7TAT, ITGA4
6rheumatoid arthritis30.7ADA, PDCD1, PNP, LBR, ITGA4
7myeloma30.6CD9, ITGA4
8non-hodgkin lymphoma30.5CT49, ITGA4, LBR, ADA, PNP, TAT
9lymphopenia30.4ADA, PNP, LBR
10kaposi's sarcoma30.2LBR, CD9, TAT
11oligodendroglioma30.2CT49
12purine nucleoside phosphorylase deficiency30.2ADA
13aseptic meningitis30.2ADA, CD9
14hepatitis c30.2CD9
15encephalitis30.2ITGA4, CD9, TAT
16acute lymphocytic leukemia30.2ADA, PNP, CD9, ITGA4
17mycosis fungoides30.2ITGA4, ADA
18schizophrenia30.2MECP2, FEZ1, LBR, POU3F1, PNP
19primary biliary cirrhosis30.2LBR
20retinoblastoma30.2TICAM2, CT49, MECP2
21dementia29.9TAT, CD9
22lupus erythematosus10.6
23sarcoidosis10.6
24chronic lymphocytic leukemia10.5
25adult syndrome10.4
26myeloid leukemia10.4
27crohn's disease10.4
28neuronitis10.4
29hodgkin's lymphoma10.4
30acute promyelocytic leukemia10.4
31acute myeloid leukemia10.3
32prostatitis10.3
33wiskott-aldrich syndrome10.3
34common variable immunodeficiency10.3
35t lymphocyte deficiency10.3
36cortical blindness10.3
37acute leukemia10.3
38central nervous system disease10.3
39meningitis10.3
40multiple myeloma10.3
41nervous system disease10.3
42psoriasis10.3
43vasculitis10.3
44rabies10.3
45breast cancer10.3
46prostate cancer10.3
47sarcoma10.3
48parotitis10.2
49nasopharyngitis10.2
50influenza10.2

Graphical network of the top 20 diseases related to Progressive Multifocal Leukoencephalopathy:



Diseases related to progressive multifocal leukoencephalopathy

Clinical Features for Progressive Multifocal Leukoencephalopathy

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Drugs & Therapeutics for Progressive Multifocal Leukoencephalopathy

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

Search CenterWatch for Progressive Multifocal Leukoencephalopathy

Drug clinical trials:

Search ClinicalTrials for Progressive Multifocal Leukoencephalopathy

Search NIH Clinical Center for Progressive Multifocal Leukoencephalopathy

Search CenterWatch for Progressive Multifocal Leukoencephalopathy

Genetic Tests for Progressive Multifocal Leukoencephalopathy

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Anatomical Context for Progressive Multifocal Leukoencephalopathy

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32MalaCards
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MalaCards organs/tissues related to Progressive Multifocal Leukoencephalopathy:

32
Brain, T cells, Kidney, Lung, Liver, B cells, Bone, Bone marrow, Spinal cord, Testes, Heart, Cortex

Animal Models for Progressive Multifocal Leukoencephalopathy or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Progressive Multifocal Leukoencephalopathy:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053909.8CARTPT, PDCD1, NFIX, CD9
2MP:00053889.6PURA, LBR, MECP2, IGHMBP2, POU3F1, CD9
3MP:00053849.2PDCD1, LBR, MECP2, ITGA4, IGBP1, PNP
4MP:00053979.2LBR, PDCD1, CARTPT, PURA, ITGA4, IGBP1
5MP:00053869.2PURA, CARTPT, FEZ1, LBR, MECP2, NFIX
6MP:00053789.1PURA, CARTPT, FEZ1, LBR, MECP2, NFIX
7MP:00107689.0MECP2, LBR, PDCD1, PURA, ITGA4, NFIX
8MP:00053878.9ADA, TICAM2, PURA, CARTPT, PDCD1, LBR
9MP:00036318.9PURA, PDCD1, FEZ1, LBR, MECP2, NFIX

Publications for Progressive Multifocal Leukoencephalopathy

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Sources:
50PubMed
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Articles related to Progressive Multifocal Leukoencephalopathy:

(show top 50)    (show all 517)
idTitleAuthorsYear
1
Progressive Multifocal Leukoencephalopathy: a rare infectious complication following allogeneic hematopoietic cell transplantation (HCT). (24118404)
2014
2
Progressive multifocal leukoencephalopathy in a patient with Good's syndrome. (23897378)
2013
3
Sequential changes in the non-coding control region sequences of JC polyomaviruses from the cerebrospinal fluid of patients with progressive multifocal leukoencephalopathy. (23138154)
2013
4
Evaluation of progressive multifocal leukoencephalopathy treatments in a Spanish cohort of HIV-infected patients: do protease inhibitors improve survival regardless of central nervous system penetration-effectiveness (CPE) score? (23217049)
2013
5
Natalizumab (tysabri)-associated progressive multifocal leukoencephalopathy: insights from perfusion magnetic resonance imaging. (24045242)
2013
6
BK virus-associated progressive multifocal leukoencephalopathy. (23451967)
2013
7
Progressive multifocal leukoencephalopathy in multiple sclerosis. (24136456)
2013
8
JC virus load in cerebrospinal fluid and transcriptional control region rearrangements may predict the clinical course of progressive multifocal leukoencephalopathy. (22253012)
2012
9
Progressive multifocal leukoencephalopathy in a patient with transitory lymphopenia. (22649215)
2012
10
Natalizumab-associated progressive multifocal leukoencephalopathy. (22931328)
2012
11
May New Biomarkers Help us to Predict Progressive Multifocal Leukoencephalopathy in HIV Positive People? (22891155)
2012
12
The pathogenesis of progressive multifocal leukoencephalopathy. (22204766)
2011
13
The changing landscape of progressive multifocal leukoencephalopathy. (21603877)
2011
14
Improved survival of HIV-1-infected patients with progressive multifocal leukoencephalopathy receiving early 5-drug combination antiretroviral therapy. (21738597)
2011
15
Progressive multifocal leukoencephalopathy (PML) development is associated with mutations in JC virus capsid protein VP1 that change its receptor specificity. (21628664)
2011
16
Progressive multifocal leukoencephalopathy in the biologic era: implications for practice. Introduction. (22123929)
2011
17
Progressive multifocal leukoencephalopathy in two psoriasis patients treated with efalizumab. (20684152)
2010
18
Role of human leukocyte antigen class I alleles in progressive multifocal leukoencephalopathy. (20105104)
2010
19
WU and KI polyomaviruses in the brains of HIV-positive patients with and without progressive multifocal leukoencephalopathy. (19860559)
2009
20
Mirtazapine in an HIV-1 infected patient with progressive multifocal leukoencephalopathy. (19359824)
2009
21
Treatment of progressive multifocal leukoencephalopathy associated with natalizumab. (19741228)
2009
22
Monoclonal antibodies and progressive multifocal leukoencephalopathy. (20073129)
2009
23
Progressive multifocal leukoencephalopathy, a review and an extended report of five patients with different immune compromised states. (19393493)
2009
24
Progressive multifocal leukoencephalopathy in a patient with undifferentiated systemic vasculitis and bilateral acute retinal necrosis. (19646359)
2009
25
5-HT2a inhibitors for progressive multifocal leukoencephalopathy: old drugs for an old disease. (18194091)
2008
26
Potential risk of progressive multifocal leukoencephalopathy with natalizumab therapy: possible interventions. (17296831)
2007
27
Hypothesis: central nervous system delivery of cyclosporine A for therapy of progressive multifocal leukoencephalopathy. (17499020)
2007
28
Longitudinal study of two cases of progressive multifocal leukoencephalopathy with a clinical benign evolution. (17613717)
2007
29
Progressive multifocal leukoencephalopathy mimicking cerebral vasculitis in systemic granulomatosis. (17052759)
2007
30
Progressive multifocal leukoencephalopathy in an immunocompetent Taiwanese patient. (17493899)
2007
31
Progressive multifocal leukoencephalopathy: lessons from AIDS and natalizumab. (16687057)
2006
32
High anti-double-stranded DNA antibodies and progressive multifocal leukoencephalopathy in a patient with systemic lupus erythematosus. (15769818)
2005
33
Macrophage chemoattractant protein-1 levels in cerebrospinal fluid correlate with containment of JC virus and prognosis of acquired immunodeficiency syndrome--associated progressive multifocal leukoencephalopathy. (16036800)
2005
34
Progressive multifocal leukoencephalopathy, natalizumab, and multiple sclerosis. (16240475)
2005
35
Progressive multifocal leukoencephalopathy and natalizumab--unforeseen consequences. (15947082)
2005
36
New insights into progressive multifocal leukoencephalopathy. (15167073)
2004
37
Detection of polyoma virus in brain tissue of patients with progressive multifocal leukoencephalopathy by real-time PCR and pyrosequencing. (15163004)
2004
38
Progressive multifocal leukoencephalopathy: diffusion-weighted imaging and pathological correlations. (14593446)
2004
39
Search for human herpesvirus 6 DNA in cerebrospinal fluid from AIDS patients with progressive multifocal leukoencephalopathy. (12587078)
2003
40
Challenges for clinical trials to treat progressive multifocal leukoencephalopathy. (12709875)
2003
41
Analysis of JC virus genotype distribution and transcriptional control region rearrangements in human immunodeficiency virus-positive progressive multifocal leukoencephalopathy patients with and without highly active antiretroviral treatment. (12709871)
2003
42
Progressive multifocal leukoencephalopathy: analysis of lesion development with diffusion-weighted MRI. (12942223)
2003
43
A pilot study of cidofovir for progressive multifocal leukoencephalopathy in AIDS. (12218391)
2002
44
Nonmyeloablative allogeneic stem cell transplantation for refractory Hodgkin's lymphoma complicated by interleukin-2 responsive progressive multifocal leukoencephalopathy. (12185517)
2002
45
Progressive multifocal leukoencephalopathy. (12084884)
2002
46
Progressive multifocal leukoencephalopathy after stem cell transplantation, unsuccessfully treated with cidofovir. (12476291)
2002
47
Expression of JC virus agnoprotein in progressive multifocal leukoencephalopathy brain. (12111355)
2002
48
Progressive multifocal leukoencephalopathy (PML) in a patient with silicosis treated previously with steroids. A report of a case with JC virus infection confirmed immunohistochemically and by electron microscopy. (10974934)
2000
49
Atypical presentations of progressive multifocal leukoencephalopathy (PML): aseptic meningitis and Anton's syndrome. (11361766)
1997
50
Primary progressive multifocal leukoencephalopathy. A central nervous system disease caused by a slow virus. (5055344)
1972

Genetic Variations for Progressive Multifocal Leukoencephalopathy

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Expression for genes affiliated with Progressive Multifocal Leukoencephalopathy

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Progressive Multifocal Leukoencephalopathy

Search GEO for disease gene expression data for Progressive Multifocal Leukoencephalopathy.

Pathways for genes affiliated with Progressive Multifocal Leukoencephalopathy

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37NCBI BioSystems Database
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Pathways related to Progressive Multifocal Leukoencephalopathy according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
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10.2ADA, PNP

Compounds for genes affiliated with Progressive Multifocal Leukoencephalopathy

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44Novoseek, 24HMDB, 11DrugBank, 59Tocris Bioscience, 28IUPHAR, 49PharmGKB
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Compounds related to Progressive Multifocal Leukoencephalopathy according to GeneCards/GeneDecks:

(show all 26)
idCompoundScoreTop Affiliating Genes
1deoxyinosine44 2411.6PNP, ADA
29-(beta-d-arabinofuranosyl)guanine4410.6ADA, PNP
3nelarabine44 1111.5ADA, PNP
4cordycepin4410.5ADA, TAT
52,3-dideoxyadenosine4410.5ADA, PNP
6pentostatin44 59 28 1113.5ADA, PNP
72,5-oligoadenylate4410.4CT49, TAT, ADA
8ribavirin44 49 1112.4LBR, PNP, ADA
9tuberculin4410.4LBR, ADA
10guanosine44 11 2412.3MECP2, TAT, PNP, ADA
11deoxyadenosine44 2411.3ADA, PNP
12neopterin4410.3LBR, CD9, ADA
13didanosine44 1111.3ADA, PNP, CD9, LBR
14vincristine44 49 1112.3ADA, TAT, ITGA4
15pentoxifylline44 1111.3LBR, ITGA4, CD9, TAT
16rantes4410.3CD9, ITGA4, LBR
17zidovudine44 1111.3LBR, CD9, TAT, ADA
18purine44 2411.2ADA, PNP, CD9, LBR
19mdms4410.2CD9, TAT
20adenosine28 11 2412.1PNP, ADA
21gold4410.0TAT, CD9, LBR
22alanine449.9CT49, MECP2, CD9, TAT, PNP, ADA
23cysteine449.8CT49, LBR, CD9, TAT, PNP, ADA
24retinoic acid44 2410.8CT49, LBR, ITGA4, CD9, TAT, ADA
25oligonucleotide449.8PURA, CT49, MECP2, CD9, TAT
26tyrosine449.2PDCD1, LBR, ITGA4, CD9, TAT, PNP

GO Terms for genes affiliated with Progressive Multifocal Leukoencephalopathy

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16Gene Ontology
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Cellular components related to Progressive Multifocal Leukoencephalopathy according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1external side of plasma membraneGO:0098979.9ADA, CD9, ITGA4, PDCD1

Biological processes related to Progressive Multifocal Leukoencephalopathy according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1purine-containing compound salvageGO:04310110.2ADA, PNP
2positive regulation of alpha-beta T cell differentiationGO:0466389.9ADA, PNP

Products for genes affiliated with Progressive Multifocal Leukoencephalopathy

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Sources for Progressive Multifocal Leukoencephalopathy

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet