MCID: CHR068
MIFTS: 37

Chronic Nk-Cell Lymphocytosis

Categories: Immune diseases

Aliases & Classifications for Chronic Nk-Cell Lymphocytosis

MalaCards integrated aliases for Chronic Nk-Cell Lymphocytosis:

Name: Chronic Nk-Cell Lymphocytosis 12 15
Chronic Lymphoproliferative Disorder of Nk-Cells 74
Nk-Cell Large Granular Lymphocyte Lymphocytosis 12

Classifications:



External Ids:

Disease Ontology 12 DOID:7465
NCIt 51 C39591
UMLS 74 C1512709

Summaries for Chronic Nk-Cell Lymphocytosis

Disease Ontology : 12 A hypersensitivity reaction type IV disease that is characterized by absolute lymphocytosis of natural killer (CD3- CD16+) cells often with concurrent cytopenia and has symptom fever, arthralgia, apthous ulcers, vasculitic skin rash, and peripheral neuropathy.

MalaCards based summary : Chronic Nk-Cell Lymphocytosis, also known as chronic lymphoproliferative disorder of nk-cells, is related to chronic lymphoproliferative disorder of natural killer cells and renal adenoma, and has symptoms including fever An important gene associated with Chronic Nk-Cell Lymphocytosis is B3GAT1 (Beta-1,3-Glucuronyltransferase 1), and among its related pathways/superpathways are Innate Immune System and ERK Signaling. The drugs Cyclophosphamide and leucovorin have been mentioned in the context of this disorder. Affiliated tissues include nk cells, skin and t cells.

Related Diseases for Chronic Nk-Cell Lymphocytosis

Diseases related to Chronic Nk-Cell Lymphocytosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 34)
# Related Disease Score Top Affiliating Genes
1 chronic lymphoproliferative disorder of natural killer cells 11.5
2 renal adenoma 10.4 B3GAT1 NCAM1
3 epithelial predominant wilms' tumor 10.3 B3GAT1 NCAM1
4 lethal midline granuloma 10.3 CD2 NCAM1
5 lymphocytic gastritis 10.3 B3GAT1 FASLG
6 pericoronitis 10.3 CD2 NCAM1
7 gastric small cell carcinoma 10.3 B3GAT1 NCAM1
8 natural killer cell leukemia 10.2 B3GAT1 CD2 NCAM1
9 inflammatory bowel disease 16 10.2 CD2 CD7
10 t-cell large granular lymphocyte leukemia 10.1 FASLG NCAM1
11 helsmoortel-van der aa syndrome 10.1 CD7 NCAM1
12 t-cell adult acute lymphocytic leukemia 10.1 CD2 CD7
13 respiratory syncytial virus infectious disease 10.1 CCL3 CCL5
14 cervix small cell carcinoma 10.1 CXCR1 NCAM1
15 chronic eosinophilic pneumonia 10.1 CCL5 ITGAM
16 acute cervicitis 10.1 CCL4 CCL5
17 japanese spotted fever 10.1 CCL4 CCL4L1
18 orofacial granulomatosis 10.0 CCL3 CCL5 CCR5
19 necatoriasis 10.0 CCL3 CCL4
20 pediatric lymphoma 10.0 B3GAT1 NCAM1
21 exposure keratitis 10.0 CCL4 CCL4L1
22 omsk hemorrhagic fever 9.9 CCL4 CCL4L1 CCL5
23 thymic dysplasia 9.9 CD2 CD7 IL15
24 stachybotrys chartarum 9.9 CCL3 CCL4 CCL5
25 sezary's disease 9.9 CD2 CD7 IL15
26 demyelinating disease 9.8 CCL3 CCL4 CCL5
27 aleukemic leukemia cutis 9.8 CD7 FUT4 NCAM1
28 whiplash 9.7 CCL3 CCL4 CCL5 CCR5
29 human immunodeficiency virus type 1 9.6 CCL3 CCL4 CCL5 CCR5 CXCR1
30 pulmonary fibrosis, idiopathic 9.6 CCL3 CCL4 CCL5 CXCR1 FASLG
31 human immunodeficiency virus infectious disease 9.5 CCL3 CCL4 CCL4L1 CCL5 CCR5
32 leukemia, acute myeloid 9.4 CD2 CD7 FUT4 ITGAM NCAM1
33 viral infectious disease 9.4 CCL3 CCL4 CCL5 CCR5 IL15
34 malaria 9.3 CCL3 CCL4 CCL5 FASLG IL15

Graphical network of the top 20 diseases related to Chronic Nk-Cell Lymphocytosis:



Diseases related to Chronic Nk-Cell Lymphocytosis

Symptoms & Phenotypes for Chronic Nk-Cell Lymphocytosis

Symptoms:

12
  • fever

Drugs & Therapeutics for Chronic Nk-Cell Lymphocytosis

Drugs for Chronic Nk-Cell Lymphocytosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 18)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
2
leucovorin Approved Phase 2 58-05-9 143 6006
3
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 126941
4
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
5 Antineoplastic Agents, Alkylating Phase 2
6 Dermatologic Agents Phase 2
7 Alkylating Agents Phase 2
8 Folic Acid Antagonists Phase 2
9 Nucleic Acid Synthesis Inhibitors Phase 2
10 Antimetabolites, Antineoplastic Phase 2
11 Immunologic Factors Phase 2
12 Vitamin B Complex Phase 2
13 Vitamin B9 Phase 2
14 Antirheumatic Agents Phase 2
15 Cyclosporins Phase 2
16 Folate Phase 2
17 Immunosuppressive Agents Phase 2
18 Antimetabolites Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Prospective, Multicentric, Phase II Randomized Controlled Trial on Two Parallel Groups Comparing the Efficacy of Two Immunosuppressive Drugs (Methotrexate, Cyclophosphamide) in Large Granular Lymphocytes Leukemia Recruiting NCT01976182 Phase 2 Methotrexate;Cyclophosphamide
2 Registry of the German CLL Study Group Recruiting NCT02863692

Search NIH Clinical Center for Chronic Nk-Cell Lymphocytosis

Genetic Tests for Chronic Nk-Cell Lymphocytosis

Anatomical Context for Chronic Nk-Cell Lymphocytosis

MalaCards organs/tissues related to Chronic Nk-Cell Lymphocytosis:

42
Nk Cells, Skin, T Cells, Cervix, Myeloid

Publications for Chronic Nk-Cell Lymphocytosis

Articles related to Chronic Nk-Cell Lymphocytosis:

# Title Authors Year
1
Chronic lymphoproliferative disorder of NK-cells: A single-institution review with emphasis on relative utility of multimodality diagnostic tools. ( 29385279 )
2018
2
Oral cyclophosphamide was effective for Coombs-negative autoimmune hemolytic anemia in CD16+CD56- chronic lymphoproliferative disorder of NK-cells. ( 28028749 )
2017
3
Clinicopathological and phenotypic features of chronic NK cell lymphocytosis identified among patients with asymptomatic lymphocytosis. ( 26234181 )
2015
4
Acute inflammatory demyelinating polyneuropathy as a manifestation of chronic lymphoproliferative disorder of NK cells. ( 25758948 )
2015
5
Activating KIRs in Chronic Lymphoproliferative Disorder of NK Cells: Protection from Viruses and Disease Induction? ( 24616720 )
2014
6
Detection of monoclonal T populations in patients with KIR-restricted chronic lymphoproliferative disorder of NK cells. ( 25193965 )
2014
7
Decreased natural killer (NK) cell function in chronic NK cell lymphocytosis associated with decreased surface expression of CD11b. ( 11286541 )
2001

Variations for Chronic Nk-Cell Lymphocytosis

Expression for Chronic Nk-Cell Lymphocytosis

Search GEO for disease gene expression data for Chronic Nk-Cell Lymphocytosis.

Pathways for Chronic Nk-Cell Lymphocytosis

Pathways related to Chronic Nk-Cell Lymphocytosis according to GeneCards Suite gene sharing:

(show all 28)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.88 CCL3 CCL4 CCL5 CCR5 CXCR1 FASLG
2
Show member pathways
13.7 CCL3 CCL4 CCL4L1 CCL5 CCR5 CD2
3
Show member pathways
13.54 CCL3 CCL4 CCL4L1 CCL5 CCR5 CD2
4
Show member pathways
13.26 CCL3 CCL4 CCL4L1 CCL5 CCR5 CXCR1
5
Show member pathways
13.22 CCL3 CCL4 CCL5 CCR5 FASLG IL15
6
Show member pathways
13.04 CCL3 CCL4 CCL4L1 CCL5 CCR5 CD2
7
Show member pathways
13 CCL3 CCL4 CCL4L1 CCL5 CCR5 CD2
8
Show member pathways
12.95 CCL3 CCL4 CCL4L1 CCL5 FASLG
9
Show member pathways
12.84 CCL3 CCL4 CCL4L1 CCL5 CCR5 FASLG
10
Show member pathways
12.76 CCL3 CCL4 CCL4L1 CCL5 CCR5 CXCR1
11 12.43 CCL5 CD2 FASLG NCAM1
12
Show member pathways
12.08 CCL3 CCL4 CCL4L1 CCL5 CCR5 CXCR1
13 11.97 CCR5 IL15 ITGAM
14
Show member pathways
11.97 CCL3 CCL4 CCL5 CD2 IL15
15 11.96 CD2 ITGAM NCAM1
16 11.89 CD2 IL15 ITGAM NCAM1
17
Show member pathways
11.87 CCL3 CCL4 CCL5 CCR5 CXCR1
18
Show member pathways
11.82 CCL3 CCL4 CCL4L1 CCR5 FASLG
19 11.77 CD2 CD7 ITGAM
20 11.73 CCL3 CCL5 IL15
21 11.7 CCL3 CCL4 CCL4L1
22 11.65 CCL3 CCL4 CCL5 IL15
23 11.56 CCL3 CCL4 CCL5
24 11.56 CCL3 CD7 IL15 ITGAM NCAM1
25 11.47 CCL3 CCL4 CCL5 CCR5
26 10.99 B3GAT1 FUT4
27 10.46 CCL3 CCL4 CCL4L1 CCL5 CD2 FASLG
28 10.4 CCL3 CCL4 CCL4L1 CCL5 CD2 FASLG

GO Terms for Chronic Nk-Cell Lymphocytosis

Cellular components related to Chronic Nk-Cell Lymphocytosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.7 CCL3 CCL4 CCL4L1 CCL5 FASLG IL15
2 external side of plasma membrane GO:0009897 9.43 CCR5 CD2 CXCR1 FASLG ITGAM NCAM1
3 cell surface GO:0009986 9.17 CCR5 CD2 FASLG FUT4 IL15 ITGAM

Biological processes related to Chronic Nk-Cell Lymphocytosis according to GeneCards Suite gene sharing:

(show all 33)
# Name GO ID Score Top Affiliating Genes
1 inflammatory response GO:0006954 9.99 CCL3 CCL4 CCL4L1 CCL5 CCR5 IL15
2 positive regulation of GTPase activity GO:0043547 9.95 CCL3 CCL4 CCL4L1 CCL5
3 regulation of signaling receptor activity GO:0010469 9.93 CCL3 CCL4 CCL5 FASLG IL15
4 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.91 CCL3 CCL4 CCL4L1 CCL5
5 cytokine-mediated signaling pathway GO:0019221 9.91 CCL3 CCL4 CCL5 CCR5 FASLG ITGAM
6 MAPK cascade GO:0000165 9.9 CCL3 CCL5 CCR5 NCAM1
7 cellular response to tumor necrosis factor GO:0071356 9.88 CCL3 CCL4 CCL4L1 CCL5
8 chemotaxis GO:0006935 9.88 CCL3 CCL4 CCL5 CCR5 CXCR1
9 cellular response to interferon-gamma GO:0071346 9.87 CCL3 CCL4 CCL4L1 CCL5
10 cell chemotaxis GO:0060326 9.86 CCL3 CCL5 CCR5 CXCR1
11 cellular response to interleukin-1 GO:0071347 9.85 CCL3 CCL4 CCL4L1 CCL5
12 calcium ion transport GO:0006816 9.83 CCL3 CCL5 CCR5
13 response to toxic substance GO:0009636 9.81 CCL3 CCL4 CCL5
14 calcium-mediated signaling GO:0019722 9.8 CCL3 CCR5 CXCR1
15 cell-cell signaling GO:0007267 9.8 CCL3 CCL4 CCL5 CCR5 FASLG IL15
16 monocyte chemotaxis GO:0002548 9.76 CCL3 CCL4 CCL4L1 CCL5
17 positive regulation of calcium ion transport GO:0051928 9.75 CCL3 CCL4 CCL5
18 dendritic cell chemotaxis GO:0002407 9.7 CCL5 CCR5 CXCR1
19 negative regulation by host of viral transcription GO:0043922 9.67 CCL3 CCL4 CCL5
20 lymphocyte chemotaxis GO:0048247 9.67 CCL3 CCL4 CCL4L1 CCL5
21 positive regulation of calcium-mediated signaling GO:0050850 9.65 CCL3 CCL4
22 neutrophil chemotaxis GO:0030593 9.65 CCL3 CCL4 CCL4L1 CCL5 CXCR1
23 regulation of T cell differentiation GO:0045580 9.64 CD2 IL15
24 macrophage chemotaxis GO:0048246 9.64 CCL3 CCL5
25 response to cholesterol GO:0070723 9.62 CCL3 CCR5
26 signaling GO:0023052 9.62 CCL3 CCR5
27 release of sequestered calcium ion into cytosol by sarcoplasmic reticulum GO:0014808 9.61 CCL3 CCR5
28 negative regulation of macrophage apoptotic process GO:2000110 9.59 CCL5 CCR5
29 positive regulation of natural killer cell chemotaxis GO:2000503 9.58 CCL3 CCL4 CCL5
30 immune response GO:0006955 9.56 CCL3 CCL4 CCL5 CCR5 CD7 CXCR1
31 eosinophil chemotaxis GO:0048245 9.46 CCL3 CCL4 CCL4L1 CCL5
32 chemokine-mediated signaling pathway GO:0070098 9.1 CCL3 CCL4 CCL4L1 CCL5 CCR5 CXCR1
33 G protein-coupled receptor signaling pathway GO:0007186 10.12 CCL3 CCL4 CCL4L1 CCL5 CCR5 CXCR1

Molecular functions related to Chronic Nk-Cell Lymphocytosis according to GeneCards Suite gene sharing:

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 cytokine activity GO:0005125 9.72 CCL3 CCL4 CCL5 FASLG IL15
2 chemokine activity GO:0008009 9.56 CCL3 CCL4 CCL4L1 CCL5
3 phosphatidylinositol phospholipase C activity GO:0004435 9.49 CCL5 CCR5
4 chemokine binding GO:0019956 9.48 CCR5 CXCR1
5 C-C chemokine binding GO:0019957 9.46 CCR5 CXCR1
6 C-C chemokine receptor activity GO:0016493 9.43 CCR5 CXCR1
7 chemokine receptor activity GO:0004950 9.4 CCR5 CXCR1
8 phospholipase activator activity GO:0016004 9.37 CCL3 CCL5
9 CCR5 chemokine receptor binding GO:0031730 9.33 CCL3 CCL4 CCL5
10 CCR1 chemokine receptor binding GO:0031726 9.13 CCL3 CCL4 CCL5
11 CCR chemokine receptor binding GO:0048020 8.92 CCL3 CCL4 CCL4L1 CCL5

Sources for Chronic Nk-Cell Lymphocytosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
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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