ANKL
MCID: AGG012
MIFTS: 43

Aggressive Nk-Cell Leukemia (ANKL)

Categories: Blood diseases, Cancer diseases, Immune diseases, Rare diseases

Aliases & Classifications for Aggressive Nk-Cell Leukemia

MalaCards integrated aliases for Aggressive Nk-Cell Leukemia:

Name: Aggressive Nk-Cell Leukemia 12 58
Natural Killer Cell Leukemia 12 15
Large Granular Lymphocyte Leukemia, Nk-Cell Type 12
Nk-Cell Large Granular Lymphocyte Leukemia 58
Aggressive Natural Killer Cell Leukemia 52
Aggressive Nk Cell Leukemia 52
Aggressive Nk-Cell Lymphoma 58
Abnormality of the Ankles 29
Nk-Cell Lgl Leukemia 58
Ankcl 58
Ankl 52

Characteristics:

Orphanet epidemiological data:

58
aggressive nk-cell leukemia
Inheritance: Multigenic/multifactorial,Not applicable; Age of onset: Adult; Age of death: adult;

Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

Disease Ontology 12 DOID:1035
NCIt 49 C8647
ICD10 32 C94.8
ICD10 via Orphanet 33 C94.7
UMLS via Orphanet 72 C1292777 C1522378
Orphanet 58 ORPHA86873
UMLS 71 C1292777

Summaries for Aggressive Nk-Cell Leukemia

Disease Ontology : 12 A lymphocytic leukemia that is characterized by the systemic proliferation of NK cells closely associated with Epstein-Barr virus and that is located in the peripheral blood, bone marrow, liver, and spleen.

MalaCards based summary : Aggressive Nk-Cell Leukemia, also known as natural killer cell leukemia, is related to composite lymphoma and large granular lymphocyte leukemia. An important gene associated with Aggressive Nk-Cell Leukemia is TIA1 (TIA1 Cytotoxic Granule Associated RNA Binding Protein), and among its related pathways/superpathways are Innate Immune System and NF-kappaB Signaling. The drugs Bortezomib and Nelfinavir have been mentioned in the context of this disorder. Affiliated tissues include peripheral blood, nk cells and bone, and related phenotypes are Reduced mammosphere formation and immune system

Wikipedia : 74 Aggressive NK-cell leukemia is a disease with an aggressive, systemic proliferation of natural killer... more...

Related Diseases for Aggressive Nk-Cell Leukemia

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

(show top 50) (show all 938)
# Related Disease Score Top Affiliating Genes
1 composite lymphoma 30.7 TIA1 GZMB
2 large granular lymphocyte leukemia 30.6 STAT3 KLRD1
3 lymphoma 30.4 TIA1 STAT3 MYC GZMB CCR5
4 pancytopenia 30.1 NCAM1 CD7 CD2
5 lichen sclerosus et atrophicus 30.0 TIA1 GZMB
6 lethal midline granuloma 30.0 TIA1 NCAM1 CD2
7 panniculitis 29.8 TIA1 NCAM1 MYC GZMB FASLG
8 t-cell lymphoma, subcutaneous panniculitis-like 29.6 TIA1 GZMB CD7
9 glomangiomatosis 29.6 NCAM1 B3GAT1
10 peripheral t-cell lymphoma 29.4 TIA1 STAT3 NCAM1 GZMB
11 acquired immunodeficiency syndrome 29.4 MYC FCGR3A CCR5
12 muscular disease 29.3 TIA1 STAT3 NCAM1 MYC
13 t-cell large granular lymphocyte leukemia 29.3 STAT3 NCAM1 KLRD1 GZMB FASLG
14 acute promyelocytic leukemia 29.3 STAT3 NCAM1 MYC CXCR1 CD7
15 leukemia, chronic myeloid 29.2 STAT3 MYC GZMB FCGR3B
16 autoimmune disease 29.2 STAT3 FCGR3B FCGR3A FASLG CCR5
17 celiac disease 1 29.1 NCAM1 KLRD1 GZMB CD7
18 leishmaniasis 28.9 GZMB FCGR3B FCGR3A CCR5
19 chronic nk-cell lymphocytosis 28.4 NCAM1 KLRD1 FCGR3B FCGR3A CD7 B3GAT1
20 inflammatory bowel disease 28.2 STAT3 MYC GZMB FCGR3B FCGR3A FASLG
21 myeloma, multiple 28.1 STAT3 NCAM1 MYC FCGR3B FCGR3A FASLG
22 lymphoma, non-hodgkin, familial 27.8 TIA1 STAT3 NCAM1 MYC GZMB FCGR3A
23 leukemia, acute myeloid 26.8 STAT3 NCAM1 MYC KLRD1 FCGR3B FCGR3A
24 leukemia, chronic lymphocytic 26.6 STAT3 NCAM1 MYC FCGR3B FCGR3A CD7
25 tenosynovitis of foot and ankle 12.3
26 arthropathy 11.9
27 hemarthrosis 11.9
28 mesomelic dysplasia, kantaputra type 11.7
29 tendinitis 11.5
30 pigmented villonodular synovitis 11.5
31 hemophilia 11.4
32 gout 11.4
33 tarsal tunnel syndrome 11.4
34 peroneal nerve paralysis 11.4
35 decubitus ulcer 11.4
36 emery-dreifuss muscular dystrophy 11.4
37 foot drop 11.4
38 exostosis 11.3
39 tendinopathy 11.3
40 clubfoot 11.3
41 osteonecrosis 11.3
42 septic arthritis 11.3
43 charcot-marie-tooth disease 11.3
44 polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy 1 11.3
45 tendinosis 11.3
46 arteriosclerosis obliterans 11.3
47 hereditary multiple osteochondromas 11.3
48 navicular bone, accessory 11.3
49 tenosynovial giant cell tumor 11.3
50 psoriatic arthritis 11.2

Graphical network of the top 20 diseases related to Aggressive Nk-Cell Leukemia:



Diseases related to Aggressive Nk-Cell Leukemia

Symptoms & Phenotypes for Aggressive Nk-Cell Leukemia

GenomeRNAi Phenotypes related to Aggressive Nk-Cell Leukemia according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Reduced mammosphere formation GR00396-S 9.1 CD7 FASLG KLRD1 MYC NCAM1 STAT3

MGI Mouse Phenotypes related to Aggressive Nk-Cell Leukemia:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 9.32 CCR5 CD7 CXCR1 FASLG FCGR3B GLIS2

Drugs & Therapeutics for Aggressive Nk-Cell Leukemia

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

(show all 8)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bortezomib Approved, Investigational Phase 1 179324-69-7 387447 93860
2
Nelfinavir Approved Phase 1 159989-64-7 64143
3
protease inhibitors Phase 1
4 HIV Protease Inhibitors Phase 1
5 Anti-Infective Agents Phase 1
6 Anti-Retroviral Agents Phase 1
7 Antiviral Agents Phase 1
8 Anti-HIV Agents Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Combined Effect of Autologous Ex-vivo Expanded Activated NK Cell-lymphocytes (ANKL) and Doublet Chemotherapy in Patients With Advanced NSCLC as the 1st Line Treatment; Phase II Recruiting NCT02370017 Phase 2
2 Phase I Trial of Nelfinavir and Bortezomib in Advanced Hematologic Malignancies Completed NCT01164709 Phase 1 bortezomib;nelfinavir mesylate
3 Study to Surveil and Track the Outcomes of Chronic Latent EBV Infection Based on Healthy Volunteers Undergoing Routine Inspection Not yet recruiting NCT03491605

Search NIH Clinical Center for Aggressive Nk-Cell Leukemia

Genetic Tests for Aggressive Nk-Cell Leukemia

Genetic tests related to Aggressive Nk-Cell Leukemia:

# Genetic test Affiliating Genes
1 Abnormality of the Ankles 29

Anatomical Context for Aggressive Nk-Cell Leukemia

The Foundational Model of Anatomy Ontology organs/tissues related to Aggressive Nk-Cell Leukemia:

19
Peripheral Blood

MalaCards organs/tissues related to Aggressive Nk-Cell Leukemia:

40
Nk Cells, Bone, T Cells, Liver, Heart, Bone Marrow, Spleen

Publications for Aggressive Nk-Cell Leukemia

Articles related to Aggressive Nk-Cell Leukemia:

(show top 50) (show all 217)
# Title Authors PMID Year
1
The CD38low natural killer cell line KHYG1 transiently expressing CD16F158V in combination with daratumumab targets multiple myeloma cells with minimal effector NK cell fratricide. 61
31919623 2020
2
Epstein - Barr virus positive T and NK-cell lymphoproliferations: Morphological features and differential diagnosis. 61
31889602 2020
3
Epstein-Barr virus NK and T cell lymphoproliferative disease: report of a 2018 international meeting. 61
31833428 2019
4
Recent advances in the diagnosis and treatment of natural killer/T-cell lymphomas. 61
31487202 2019
5
[Clinical research of HLA-haploidentical peripheral hematopoietic stem cell transplantation following reduced intensity conditioning regimen with hematological malignancy patients over 50 years old]. 61
31495134 2019
6
Frequent structural variations involving programmed death ligands in Epstein-Barr virus-associated lymphomas. 61
30683910 2019
7
Successful Treatment of Pediatric Epstein-Barr Virus-positive Aggressive Natural Killer-Cell Leukemia. 61
29889803 2019
8
Identification of a novel CCDC22 mutation in a patient with severe Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis and aggressive natural killer cell leukemia. 61
30706328 2019
9
Analysis of preventability of malignancy-related maternal death from the nationwide registration system of maternal deaths in Japan. 61
30999803 2019
10
Determination of Epstein-Barr Virus-Infected Lymphocyte Cell Types in Peripheral Blood Mononuclear Cells as a Valuable Diagnostic Tool in Hematological Diseases. 61
31065564 2019
11
Epstein-Barr Virus-Associated T and NK-Cell Lymphoproliferative Diseases. 61
30931288 2019
12
Somatic mutations in KMT2D and TET2 associated with worse prognosis in Epstein-Barr virus-associated T or natural killer-cell lymphoproliferative disorders. 61
31311407 2019
13
Cyclin-dependent kinase 9 as a potential specific molecular target in NK-cell leukemia/lymphoma. 61
30076184 2018
14
Allogeneic Stem Cell Transplantation for Patients with Natural Killer/T Cell Lymphoid Malignancy: A Multicenter Analysis Comparing Upfront and Salvage Transplantation. 61
30064012 2018
15
[Precision genomic and translational medicine for acute myeloid leukemia]. 61
30465531 2018
16
Large granular lymphocytic leukemia CD3-CD56-: a challenge for the biologist and the physician. 61
30078779 2018
17
Expression of activating natural killer-cell receptors is a hallmark of the innate-like T-cell neoplasm in peripheral T-cell lymphomas. 61
29363227 2018
18
Aggressive natural killer-cell leukemia mutational landscape and drug profiling highlight JAK-STAT signaling as therapeutic target. 61
29674644 2018
19
Integrated genomic analysis identifies deregulated JAK/STAT-MYC-biosynthesis axis in aggressive NK-cell leukemia. 61
29148541 2018
20
The utility of flow cytometry in differentiating NK/T cell lymphoma from indolent and reactive NK cell proliferations. 61
28431200 2018
21
Aggressive NK-Cell Leukemia. 61
30364049 2018
22
EBV in T-/NK-Cell Tumorigenesis. 61
29896680 2018
23
Overview of EBV-Associated T/NK-Cell Lymphoproliferative Diseases. 61
30662890 2018
24
Transcriptomic Abnormalities in Epstein Barr Virus Associated T/NK Lymphoproliferative Disorders. 61
30705877 2018
25
Aggressive natural killer cell leukemia mimicking interstitial lung diseases with the activation of the mitogen-activated protein kinase pathway. 61
31949637 2018
26
[Aggressive natural killer cell leukemia: study of a case occurred in Africa and literature review]. 61
30918555 2018
27
Combination statin and chemotherapy inhibits proliferation and cytotoxicity of an aggressive natural killer cell leukemia. 61
30116531 2018
28
Aggressive NK-cell leukemia: clinical subtypes, molecular features, and treatment outcomes. 61
29263371 2017
29
EBV-negative aggressive NK-cell leukemia/lymphoma: a clinical and pathological study from a single institution. 61
28548121 2017
30
Clinical analysis of 42 cases of EBV-positive mature T/NK-cell neoplasms. 61
28672968 2017
31
Aggressive natural killer cell leukemia or extranodal NK/T cell lymphoma? a case with nasal involvement. 61
28623913 2017
32
Allogeneic Hematopoietic Cell Transplantation for Aggressive NK Cell Leukemia. A Center for International Blood and Marrow Transplant Research Analysis. 61
28161608 2017
33
Achievement of disease control with donor-derived EB virus-specific cytotoxic T cells after allogeneic peripheral blood stem cell transplantation for aggressive NK-cell leukemia. 61
27830541 2017
34
Clinical Features, Pathogenesis, and Treatment of Large Granular Lymphocyte Leukemias. 61
28717070 2017
35
A screening method with lymphocyte percentage and proportion of granular lymphocytes in the peripheral blood for large granular lymphocyte (LGL) leukemia. 61
27686672 2017
36
EBV induces persistent NF-κB activation and contributes to survival of EBV-positive neoplastic T- or NK-cells. 61
28346502 2017
37
CD56-Negative Aggressive NK Cell Leukemia Relapsing as Multiple Cranial Nerve Palsies: Case Report and Literature Review. 61
29163992 2017
38
EBV-negative Aggressive NK-cell Leukemia/Lymphoma: Clinical, Pathologic, and Genetic Features. 61
27631517 2017
39
Aggressive natural killer cell leukemia: a case report. 61
29263706 2017
40
[The understanding of Epstein-Barr virus associated lymphoproliferative disorder]. 61
28056294 2016
41
Aggressive natural killer cell leukemia associated with hemophagocytic lymphohistiocytosis 61
28829111 2016
42
Aggressive natural killer cell leukemia associated with hemophagocytic lymphohistiocytosis. 61
28829754 2016
43
Clinical features and treatment outcome of Epstein-Barr virus-positive nodal T-cell lymphoma. 61
27456462 2016
44
A Pyrazolo[3,4-d]pyrimidine compound inhibits Fyn phosphorylation and induces apoptosis in natural killer cell leukemia. 61
27566560 2016
45
Clinicopathologic Characterization of Aggressive Natural Killer Cell Leukemia Involving Different Tissue Sites. 61
26975038 2016
46
Cell size variations of large granular lymphocyte leukemia: Implication of a small cell subtype of granular lymphocyte leukemia with STAT3 mutations. 61
27064362 2016
47
Nasal-type NK/T-cell lymphomas are more frequently T rather than NK lineage based on T-cell receptor gene, RNA, and protein studies: lineage does not predict clinical behavior. 61
27015135 2016
48
EBV-associated aggressive natural killer cell leukemia. 61
28092886 2016
49
L-asparaginase-based regimens followed by allogeneic hematopoietic stem cell transplantation improve outcomes in aggressive natural killer cell leukemia. 61
27091029 2016
50
A relapsed aggressive NK-cell leukemia with CNS involvement diagnosed by cerebrospinal fluid examination. 61
26890009 2016

Variations for Aggressive Nk-Cell Leukemia

Expression for Aggressive Nk-Cell Leukemia

Search GEO for disease gene expression data for Aggressive Nk-Cell Leukemia.

Pathways for Aggressive Nk-Cell Leukemia

Pathways related to Aggressive Nk-Cell Leukemia according to GeneCards Suite gene sharing:

(show all 11)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.74 STAT3 NCAM1 MYC KLRD1 FCGR3B FCGR3A
2 12.23 STAT3 NCAM1 GZMB FASLG CD2
3
Show member pathways
12.13 KLRD1 GZMB FCGR3B FCGR3A FASLG
4
Show member pathways
12.01 STAT3 FCGR3B FCGR3A CCR5
5 11.97 TIA1 MYC GZMB FASLG
6 11.78 STAT3 NCAM1 MYC
7 11.69 STAT3 MYC FASLG
8
Show member pathways
11.51 STAT3 GZMB FASLG CCR5
9 11.16 NCAM1 KLRD1 GZMB CD7
10 11.09 TIA1 STAT3 MYC
11 10.63 STAT3 MYC FASLG CXCR1

GO Terms for Aggressive Nk-Cell Leukemia

Cellular components related to Aggressive Nk-Cell Leukemia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 integral component of membrane GO:0016021 9.7 NCAM1 MYC KLRD1 FCGR3B FCGR3A FASLG
2 external side of plasma membrane GO:0009897 9.17 NCAM1 KLRD1 FCGR3A FASLG CXCR1 CD2

Biological processes related to Aggressive Nk-Cell Leukemia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytokine-mediated signaling pathway GO:0019221 9.46 STAT3 MYC FASLG CCR5
2 dendritic cell chemotaxis GO:0002407 9.32 CXCR1 CCR5
3 response to growth factor GO:0070848 9.26 MYC FASLG
4 cell surface receptor signaling pathway GO:0007166 9.26 KLRD1 CXCR1 CD2 CCR5
5 immune response GO:0006955 9.1 FCGR3B FCGR3A FASLG CXCR1 CD7 CCR5

Molecular functions related to Aggressive Nk-Cell Leukemia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 chemokine binding GO:0019956 9.32 CXCR1 CCR5
2 C-C chemokine binding GO:0019957 9.26 CXCR1 CCR5
3 C-C chemokine receptor activity GO:0016493 9.16 CXCR1 CCR5
4 chemokine receptor activity GO:0004950 8.96 CXCR1 CCR5
5 IgG binding GO:0019864 8.62 FCGR3B FCGR3A

Sources for Aggressive Nk-Cell Leukemia

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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