ANKL
MCID: AGG012
MIFTS: 44

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 15
Large Granular Lymphocyte Leukemia, Nk-Cell Type 12
Nk-Cell Large Granular Lymphocyte Leukemia 58
Aggressive Natural Killer Cell Leukemia 52
Natural Killer Cell Leukaemia 12
Aggressive Nk-Cell Leukaemia 12
Natural Killer Cell Leukemia 12
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
SNOMED-CT 67 721310007
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 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 large granular lymphocyte leukemia, nk-cell type, is related to large granular lymphocyte leukemia and adult t-cell 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 NF-kappaB Signaling and Immune response Role of DAP12 receptors in NK cells. The drugs Nelfinavir and Bortezomib have been mentioned in the context of this disorder. Affiliated tissues include peripheral blood, nk cells and t cells, and related phenotypes are Reduced mammosphere formation and hematopoietic 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 956)
# Related Disease Score Top Affiliating Genes
1 large granular lymphocyte leukemia 30.3 STAT3 KLRD1
2 adult t-cell leukemia 30.2 STAT3 MYC CD7
3 composite lymphoma 30.2 TIA1 GZMB CD5
4 lichen sclerosus et atrophicus 30.0 TIA1 GZMB
5 t-cell lymphoblastic leukemia/lymphoma 30.0 STAT3 MYC FASLG CD7
6 panniculitis 29.9 TIA1 NCAM1 MYC GZMB FASLG
7 pancytopenia 29.9 NCAM1 CD7 CD5 CD2
8 lymphoma 29.9 TIA1 STAT3 MYC GZMB CD5 CCR5
9 glomangiomatosis 29.8 NCAM1 B3GAT1
10 lymphoblastic lymphoma 29.7 MYC CD7 CD5
11 t-cell lymphoma, subcutaneous panniculitis-like 29.7 TIA1 GZMB CD7
12 multiple sclerosis 29.6 STAT3 FASLG CXCR1 CD2 CCR5
13 t-cell adult acute lymphocytic leukemia 29.5 TIA1 CD7 CD5 CD2
14 lethal midline granuloma 29.5 TIA1 NCAM1 GZMB CD7 CD2
15 acute promyelocytic leukemia 29.3 STAT3 NCAM1 MYC CXCR1 CD7
16 b-cell lymphoma 29.3 TIA1 STAT3 MYC CD5
17 autoimmune disease 29.3 STAT3 FCGR3B FCGR3A FASLG CCR5
18 acquired immunodeficiency syndrome 29.2 MYC FCGR3A CCR5
19 peripheral t-cell lymphoma 28.9 TIA1 STAT3 NCAM1 GZMB CD5
20 leishmaniasis 28.9 GZMB FCGR3B FCGR3A CCR5
21 follicular lymphoma 28.8 MYC FCGR3A CD5
22 leukemia, chronic myeloid 28.6 STAT3 MYC GZMB FCGR3B
23 celiac disease 1 28.6 TIA1 NCAM1 KLRD1 GZMB CD7
24 t-cell large granular lymphocyte leukemia 28.6 STAT3 NCAM1 KLRD1 GZMB FASLG B3GAT1
25 chronic nk-cell lymphocytosis 28.4 NCAM1 KLRD1 FCGR3B FCGR3A CD7 B3GAT1
26 immune deficiency disease 28.4 FCGR3B FCGR3A FASLG CD5 CD2 CCR5
27 inflammatory bowel disease 28.3 STAT3 MYC GZMB FCGR3B FCGR3A FASLG
28 lymphoma, non-hodgkin, familial 28.0 STAT3 NCAM1 MYC GZMB FCGR3A CD7
29 myeloma, multiple 27.8 STAT3 NCAM1 MYC GZMB FCGR3B FCGR3A
30 leukemia, chronic lymphocytic 26.9 STAT3 NCAM1 MYC FCGR3B FCGR3A CD7
31 leukemia, acute myeloid 26.1 STAT3 NCAM1 MYC KLRD1 FCGR3B FCGR3A
32 tenosynovitis of foot and ankle 12.3
33 arthropathy 11.9
34 hemarthrosis 11.9
35 mesomelic dysplasia, kantaputra type 11.7
36 tendinitis 11.5
37 pigmented villonodular synovitis 11.5
38 hemophilia 11.4
39 gout 11.4
40 tarsal tunnel syndrome 11.4
41 peroneal nerve paralysis 11.4
42 decubitus ulcer 11.4
43 emery-dreifuss muscular dystrophy 11.4
44 foot drop 11.4
45 exostosis 11.3
46 tendinopathy 11.3
47 clubfoot 11.3
48 osteonecrosis 11.3
49 septic arthritis 11.3
50 charcot-marie-tooth disease 11.3

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 9.17 CD5 CD7 FASLG KLRD1 MYC NCAM1

MGI Mouse Phenotypes related to Aggressive Nk-Cell Leukemia:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.61 CCR5 CD5 CD7 CXCR1 FASLG FCGR3B
2 immune system MP:0005387 9.32 CCR5 CD5 CD7 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
Nelfinavir Approved Phase 1 159989-64-7 64143
2
Bortezomib Approved, Investigational Phase 1 179324-69-7 387447 93860
3 Anti-HIV Agents Phase 1
4
protease inhibitors Phase 1
5 Anti-Infective Agents Phase 1
6 HIV Protease Inhibitors Phase 1
7 Anti-Retroviral Agents Phase 1
8 Antiviral 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 Unknown status 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 Recruiting NCT03491605
4 Blastic Plasmacytoid Dendritic Cell Neoplasm in Korean Population: A Multicenter Study Active, not recruiting NCT03974971

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, T Cells, Bone Marrow, Bone, Spleen, Liver, Myeloid

Publications for Aggressive Nk-Cell Leukemia

Articles related to Aggressive Nk-Cell Leukemia:

(show top 50) (show all 125)
# Title Authors PMID Year
1
Epstein-Barr virus NK and T cell lymphoproliferative disease: report of a 2018 international meeting. 61
31833428 2020
2
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
3
Epstein - Barr virus positive T and NK-cell lymphoproliferations: Morphological features and differential diagnosis. 61
31889602 2020
4
Insights Into Genetic Landscape of Large Granular Lymphocyte Leukemia. 61
32133291 2020
5
Recent advances in the diagnosis and treatment of natural killer/T-cell lymphomas. 61
31487202 2019
6
Frequent structural variations involving programmed death ligands in Epstein-Barr virus-associated lymphomas. 61
30683910 2019
7
Analysis of preventability of malignancy-related maternal death from the nationwide registration system of maternal deaths in Japan. 61
30999803 2019
8
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
9
Epstein-Barr Virus-Associated T and NK-Cell Lymphoproliferative Diseases. 61
30931288 2019
10
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
11
Non-EBV-Related Aggressive NK-Cell Leukemia: An Oncohematological Great Imitator. 61
32300433 2018
12
Cyclin-dependent kinase 9 as a potential specific molecular target in NK-cell leukemia/lymphoma. 61
30076184 2018
13
Allogeneic Stem Cell Transplantation for Patients with Natural Killer/T Cell Lymphoid Malignancy: A Multicenter Analysis Comparing Upfront and Salvage Transplantation. 61
30064012 2018
14
[Precision genomic and translational medicine for acute myeloid leukemia]. 61
30465531 2018
15
Large granular lymphocytic leukemia CD3-CD56-: a challenge for the biologist and the physician. 61
30078779 2018
16
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
17
Integrated genomic analysis identifies deregulated JAK/STAT-MYC-biosynthesis axis in aggressive NK-cell leukemia. 61
29148541 2018
18
EBV in T-/NK-Cell Tumorigenesis. 61
29896680 2018
19
The utility of flow cytometry in differentiating NK/T cell lymphoma from indolent and reactive NK cell proliferations. 61
28431200 2018
20
Aggressive NK-Cell Leukemia. 61
30364049 2018
21
Overview of EBV-Associated T/NK-Cell Lymphoproliferative Diseases. 61
30662890 2018
22
Transcriptomic Abnormalities in Epstein Barr Virus Associated T/NK Lymphoproliferative Disorders. 61
30705877 2018
23
Aggressive NK-cell leukemia: clinical subtypes, molecular features, and treatment outcomes. 61
29263371 2017
24
EBV-negative aggressive NK-cell leukemia/lymphoma: a clinical and pathological study from a single institution. 61
28548121 2017
25
Clinical analysis of 42 cases of EBV-positive mature T/NK-cell neoplasms. 61
28672968 2017
26
Allogeneic Hematopoietic Cell Transplantation for Aggressive NK Cell Leukemia. A Center for International Blood and Marrow Transplant Research Analysis. 61
28161608 2017
27
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
28
LGL leukemia: from pathogenesis to treatment. 61
28115367 2017
29
CD56-Negative Aggressive NK Cell Leukemia Relapsing as Multiple Cranial Nerve Palsies: Case Report and Literature Review. 61
29163992 2017
30
EBV-negative Aggressive NK-cell Leukemia/Lymphoma: Clinical, Pathologic, and Genetic Features. 61
27631517 2017
31
EBV induces persistent NF-κB activation and contributes to survival of EBV-positive neoplastic T- or NK-cells. 61
28346502 2017
32
Clinical Features, Pathogenesis, and Treatment of Large Granular Lymphocyte Leukemias. 61
28717070 2017
33
A screening method with lymphocyte percentage and proportion of granular lymphocytes in the peripheral blood for large granular lymphocyte (LGL) leukemia. 61
27686672 2017
34
[The understanding of Epstein-Barr virus associated lymphoproliferative disorder]. 61
28056294 2016
35
Clinical features and treatment outcome of Epstein-Barr virus-positive nodal T-cell lymphoma. 61
27456462 2016
36
Cell size variations of large granular lymphocyte leukemia: Implication of a small cell subtype of granular lymphocyte leukemia with STAT3 mutations. 61
27064362 2016
37
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
38
A relapsed aggressive NK-cell leukemia with CNS involvement diagnosed by cerebrospinal fluid examination. 61
26890009 2016
39
[Large Granular Lymphocytic Leukemia and JAK/STAT Signaling Pathway--Review]. 61
26913432 2016
40
A case of lymphoproliferative disorder of NK-cells: aggressive immunophenotype but indolent behavior. 61
26401278 2015
41
Aggressive natural killer-cell neoplasm presenting in the marrow: a report of two cases including one with gains of chromosomes 4q and 9p. 61
26141723 2015
42
Aggressive mature natural killer cell neoplasms: report on a series of 12 European patients with emphasis on flow cytometry based immunophenotype and DNA content of neoplastic natural killer cells. 61
24669939 2015
43
Aggressive NK-cell leukemia in a 69 years old Caucasian woman: a case report. 61
26682116 2015
44
Prognostic implication of morphology, cyclinE2 and proliferation in EBV-associated T/NK lymphoproliferative disease in non-immunocompromised hosts. 61
25475054 2014
45
Cutaneous intravascular natural killer-cell lymphoma: a case report and review of the literature. 61
25015867 2014
46
De novo CD3 negative hepatosplenic T-cell lymphoma: diagnostic challenges and pitfalls. 61
24978925 2014
47
[Large granular lymphocyte leukemia]. 61
24989304 2014
48
Abnormal immunophenotype provides a key diagnostic marker: a report of 29 cases of de novo aggressive natural killer cell leukemia. 61
24524877 2014
49
18F-FDG PET for diagnosis and response assessment for aggressive NK cell leukemia. 61
23751830 2014
50
[Diagnosis and differential diagnosis of aggressive NK cell leukemia]. 61
24759008 2014

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 12)
# Super pathways Score Top Affiliating Genes
1 12.23 STAT3 NCAM1 GZMB FASLG CD2
2
Show member pathways
12.13 KLRD1 GZMB FCGR3B FCGR3A FASLG
3
Show member pathways
12.01 STAT3 FCGR3B FCGR3A CCR5
4 11.97 TIA1 MYC GZMB FASLG
5 11.81 STAT3 NCAM1 MYC
6 11.74 NCAM1 CD5 CD2
7 11.71 STAT3 MYC FASLG
8 11.63 CD7 CD5 CD2
9
Show member pathways
11.51 STAT3 GZMB FASLG CCR5
10 11.16 NCAM1 KLRD1 GZMB CD7
11 11.09 TIA1 STAT3 MYC
12 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 membrane GO:0016020 10.07 NCAM1 MYC KLRD1 GZMB FCGR3B FCGR3A
2 plasma membrane GO:0005886 9.9 STAT3 NCAM1 KLRD1 FCGR3B FCGR3A FASLG
3 integral component of membrane GO:0016021 9.73 NCAM1 MYC KLRD1 FCGR3B FCGR3A FASLG
4 external side of plasma membrane GO:0009897 9.23 NCAM1 KLRD1 FCGR3A FASLG CXCR1 CD5

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 immune response GO:0006955 9.43 FCGR3B FCGR3A FASLG CXCR1 CD7 CCR5
3 dendritic cell chemotaxis GO:0002407 9.37 CXCR1 CCR5
4 response to growth factor GO:0070848 9.32 MYC FASLG
5 positive regulation of ATP biosynthetic process GO:2001171 9.26 STAT3 MYC
6 cell surface receptor signaling pathway GO:0007166 9.1 KLRD1 FCGR3B FCGR3A CXCR1 CD2 CCR5

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

# Name GO ID Score Top Affiliating Genes
1 C-C chemokine binding GO:0019957 9.26 CXCR1 CCR5
2 C-C chemokine receptor activity GO:0016493 9.16 CXCR1 CCR5
3 chemokine receptor activity GO:0004950 8.96 CXCR1 CCR5
4 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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