ICL
MCID: IDP001
MIFTS: 28

Idiopathic Cd4-Positive T-Lymphocytopenia (ICL)

Categories: Blood diseases, Immune diseases, Rare diseases

Aliases & Classifications for Idiopathic Cd4-Positive T-Lymphocytopenia

MalaCards integrated aliases for Idiopathic Cd4-Positive T-Lymphocytopenia:

Name: Idiopathic Cd4-Positive T-Lymphocytopenia 12 15 17
T-Lymphocytopenia, Idiopathic Cd4-Positive 43 71
Immunodeficiency 13 52 71
Idiopathic Cd4 Positive T-Lymphocytopenia 52
Idiopathic Cd4 Lymphocytopenia 52
Idiopathic Cd4 Lymphopenia 52
Imd13 52
Icl 52

Classifications:



External Ids:

Disease Ontology 12 DOID:3109
MeSH 43 D018344
NCIt 49 C84780
UMLS 71 C0206744 C3809768

Summaries for Idiopathic Cd4-Positive T-Lymphocytopenia

NIH Rare Diseases : 52 Idiopathic CD4 positive T-lymphocytopenia (ICL) is a rare disorder of the immune system . People with ICL have low levels of a type of white blood cell , called a CD4+ T cell . These low levels can not be explained by other causes of immunodeficiency , including HIV infection . T cells have many jobs in our immune system, such as attacking bacteria and viruses . CD4 is a protein found on the surface of many different cells within your immune system. It lets the different cells of your immune system work with each other. When CD4+ T cells are decreased, your body becomes more prone to infection. Signs and Symptoms of ICL vary. Some people have no symptoms, however most have illnesses suggestive of a lowered immune system, including infections (varicella-zoster virus , human papilloma virus ), autoimmune disorders (autoimmune hemolytic anemia , lupus ), and certain types of cancer (non-Hodgkin lymphoma ). A few people with ICL are found to carry specific gene mutations ; however, for most cases of ICL the underlying cause is not known. Currently, there is no cure for ICL, but treatments are available to help manage individual symptoms.

MalaCards based summary : Idiopathic Cd4-Positive T-Lymphocytopenia, also known as t-lymphocytopenia, idiopathic cd4-positive, is related to acquired immunodeficiency syndrome and immune deficiency disease. An important gene associated with Idiopathic Cd4-Positive T-Lymphocytopenia is BCL6 (BCL6 Transcription Repressor), and among its related pathways/superpathways are B Cell Receptor Signaling Pathway (sino) and B Cell Development Pathways. The drugs Neurotransmitter Agents and Immunologic Factors have been mentioned in the context of this disorder. Affiliated tissues include t cells, b cells and lymph node.

Disease Ontology : 12 A lymphopenia that is caused by a reduction of CD4+ T lymphocytes.

Related Diseases for Idiopathic Cd4-Positive T-Lymphocytopenia

Diseases related to Idiopathic Cd4-Positive T-Lymphocytopenia via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 155)
# Related Disease Score Top Affiliating Genes
1 acquired immunodeficiency syndrome 29.7 CD4 BCL6
2 immune deficiency disease 29.4 CD4 BCL6
3 lymphoma, non-hodgkin, familial 29.2 CD4 BCL6
4 leukemia, acute myeloid 28.8 CD4 BCL6
5 immunodeficiency 13 12.6
6 cutaneous lupus erythematosus 11.2
7 myopia 10.7
8 astigmatism 10.6
9 progressive multifocal leukoencephalopathy 10.5
10 refractive error 10.5
11 lymphopenia 10.4
12 keratoconus 10.4
13 autoimmune disease 10.3
14 chickenpox 10.3
15 fanconi anemia, complementation group a 10.3
16 ocular motor apraxia 10.3
17 candidiasis 10.3
18 histoplasmosis 10.3
19 demyelinating disease 10.3
20 mucormycosis 10.3
21 cryptococcal meningitis 10.3
22 meningitis 10.3
23 astrocytoma 10.2
24 grade iii astrocytoma 10.2
25 cataract 10.2
26 kaposi sarcoma 10.2
27 ataxia and polyneuropathy, adult-onset 10.2
28 diffuse large b-cell lymphoma 10.2
29 neuroretinitis 10.2
30 retinitis 10.2
31 b-cell lymphoma 10.2
32 molluscum contagiosum 10.2
33 lymphoma aids related 10.2
34 cryptococcosis 10.2
35 intraocular pressure quantitative trait locus 10.1
36 immunodeficiency-centromeric instability-facial anomalies syndrome 3 10.1
37 deficiency anemia 10.1
38 suppression amblyopia 10.1
39 amblyopia 10.1
40 presbyopia 10.1
41 anisometropia 10.1
42 acute closed-angle glaucoma 10.1
43 myositis 10.0
44 thrombocytopenic purpura, autoimmune 10.0
45 lymphoma, hodgkin, classic 10.0
46 mycosis fungoides 10.0
47 retinitis pigmentosa 10.0
48 sjogren syndrome 10.0
49 aceruloplasminemia 10.0
50 kala-azar 1 10.0

Graphical network of the top 20 diseases related to Idiopathic Cd4-Positive T-Lymphocytopenia:



Diseases related to Idiopathic Cd4-Positive T-Lymphocytopenia

Symptoms & Phenotypes for Idiopathic Cd4-Positive T-Lymphocytopenia

Drugs & Therapeutics for Idiopathic Cd4-Positive T-Lymphocytopenia

Drugs for Idiopathic Cd4-Positive T-Lymphocytopenia (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 14)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Neurotransmitter Agents Phase 2
2 Immunologic Factors Phase 2
3 Adjuvants, Immunologic Phase 2
4 Anticonvulsants Phase 2
5 Pyridostigmine Bromide Phase 2 101-26-8
6 Cholinergic Agents Phase 2
7 Bromides Phase 2
8 Cholinesterase Inhibitors Phase 2
9
belimumab Approved Phase 1 356547-88-1 5957 10451420
10 Interleukin-4 Phase 1
11 Immunosuppressive Agents Phase 1
12 Autoantibodies Phase 1
13
Adenosine Approved, Investigational 58-61-7 60961
14
carbamide peroxide Approved 124-43-6

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Study of the Role of Peripheral Acetylcholinesterase Inhibitor Pyridostigmine as Immunomodulators in a Population of Patients Living With Human Immunodeficiency Virus Infection. Enrolling by invitation NCT03312244 Phase 2 Pyridostigmine Bromide;Placebo
2 Interleukin-7 (CYT107) Treatment of Idiopathic CD4 Lymphocytopenia: Expansion of CD4 T Cells (ICICLE) Terminated NCT00839436 Phase 1, Phase 2 CYT107
3 A Phase I Evaluation of the Safety of Belimumab in People With Idiopathaic CD4 Lymphopenia and Autoantibodies (Phoebe) Recruiting NCT04097561 Phase 1
4 Regional Differences in Human Immunodeficiency Virus (HIV) Testing Procedures in Eastern German Federal States Unknown status NCT03158987
5 Evaluation of the Epidemiology, Clinical Manifestations, Etiologies, and Immunology of Human Immunodeficiency Virus (HIV) Negative CD4 T Lymphocyte Deficient Patients Completed NCT00001319
6 Analysis of Clinical and Immunological Characteristics, as Well as Pathophysiological Mechanisms in a French Cohort of Patients With Idiopathic CD4 Lymphocytopenia Completed NCT02113930
7 Etiology, Pathogenesis, and Natural History of Idiopathic CD4+ Lymphocytopenia Recruiting NCT00867269
8 Hematopoietic Stem Cell Mobilization in Idiopathic CD4 Lymphocytopenia Patients and Healthy Controls for the Study of T Cell Maturation and Trafficking in Murine Models Recruiting NCT02015013
9 Virologic and Immunologic Evaluation of Lymph Node, Tonsillar, and Intestinal Biopsies, and Bronchoalveolar Lavage Fluid Recruiting NCT00001471

Search NIH Clinical Center for Idiopathic Cd4-Positive T-Lymphocytopenia

Cochrane evidence based reviews: t-lymphocytopenia, idiopathic cd4-positive

Genetic Tests for Idiopathic Cd4-Positive T-Lymphocytopenia

Anatomical Context for Idiopathic Cd4-Positive T-Lymphocytopenia

MalaCards organs/tissues related to Idiopathic Cd4-Positive T-Lymphocytopenia:

40
T Cells, B Cells, Lymph Node, Testes, Spinal Cord, Lung, Cervix

Publications for Idiopathic Cd4-Positive T-Lymphocytopenia

Articles related to Idiopathic Cd4-Positive T-Lymphocytopenia:

# Title Authors PMID Year
1
Multiple flat warts associated with idiopathic CD4-positive T lymphocytopenia. 61
18191700 2008
2
Human herpes virus 8-negative primary effusion lymphoma with BCL6 rearrangement in a patient with idiopathic CD4 positive T-lymphocytopenia. 61
18166773 2008
3
Pleural cryptococcosis with idiopathic CD4 positive T-lymphocytopenia. 61
18827437 2008
4
Pleural cryptococcosis with idiopathic CD4 positive T-lymphocytopenia. 61
15575251 2004
5
Intracranial dissemination from thoracic spinal cord anaplastic astrocytoma in a patient with idiopathic CD4-positive T lymphocytopenia: a case report. 61
11546571 2001

Variations for Idiopathic Cd4-Positive T-Lymphocytopenia

Expression for Idiopathic Cd4-Positive T-Lymphocytopenia

Search GEO for disease gene expression data for Idiopathic Cd4-Positive T-Lymphocytopenia.

Pathways for Idiopathic Cd4-Positive T-Lymphocytopenia

Pathways related to Idiopathic Cd4-Positive T-Lymphocytopenia according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.29 CD4 BCL6
2 10.35 CD4 BCL6

GO Terms for Idiopathic Cd4-Positive T-Lymphocytopenia

Biological processes related to Idiopathic Cd4-Positive T-Lymphocytopenia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 immune system process GO:0002376 8.96 CD4 BCL6
2 cytokine-mediated signaling pathway GO:0019221 8.62 CD4 BCL6

Sources for Idiopathic Cd4-Positive T-Lymphocytopenia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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