MCID: BLS007
MIFTS: 45

Blastic Plasmacytoid Dendritic Cell malady

Rare diseases, Cancer diseases, Blood diseases, Immune diseases categories

Summaries for Blastic Plasmacytoid Dendritic Cell

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MalaCards based summary: Blastic Plasmacytoid Dendritic Cell, also known as t-cell prolymphocytic leukemia, is related to chronic lymphocytic leukemia and myeloid leukemia. An important gene associated with Blastic Plasmacytoid Dendritic Cell is TET2 (tet methylcytosine dioxygenase 2), and among its related pathways are Notch-mediated HES/HEY network and Malaria. The compounds [alcl] and hydralazine have been mentioned in the context of this disorder. Affiliated tissues include t cells, myeloid and skin, and related mouse phenotypes are tumorigenesis and no phenotypic analysis.

Aliases & Classifications for Blastic Plasmacytoid Dendritic Cell

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Sources:
41NIH Rare Diseases, 43Novoseek, 47Orphanet, 60UMLS, 34MESH via Orphanet, 26ICD10 via Orphanet, 61UMLS via Orphanet, 25ICD10
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Blastic Plasmacytoid Dendritic Cell, Aliases & Descriptions:

Name: Blastic Plasmacytoid Dendritic Cell 41
T-Cell Prolymphocytic Leukemia 41 47 60
Blastic Plasmacytoid Dendritic Cell Neoplasm 41 60
T-Cell Chronic Lymphocytic Leukemia 41 47
 
T-Pll 41 47
Lymphocytic Leukemia T-Cell Chronic 43
Leukemia, T-Cell, Chronic 60
Bpdcn 41


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases, Cancer diseases
Anatomical: Blood diseases, Immune diseases


Characteristics (Orphanet epidemiological data):

47
t-cell prolymphocytic leukemia:
Age of onset: Adult


External Ids:

Orphanet47 86871
MESH via Orphanet34 D015461
ICD10 via Orphanet26 C91.3
UMLS via Orphanet61 C2363142
ICD1025 C91.6

Related Diseases for Blastic Plasmacytoid Dendritic Cell

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Diseases related to Blastic Plasmacytoid Dendritic Cell via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50)    (show all 81)
idRelated DiseaseScoreTop Affiliating Genes
1chronic lymphocytic leukemia31.4TCL1A, IL3RA, CD4
2myeloid leukemia31.2NPM1, MPO, IL3RA
3myeloid sarcoma30.7NCAM1, MPO
4b-cell lymphomas30.4CDKN1B, NPM1, TCL1A, CDKN2A
5acute leukemia30.2MPO, NPM1, NCAM1, IL3RA
6leukemia29.2TCL1A, TET2, SPIB, PECAM1, MPO, CD4
7breast cancer29.1PECAM1, NPM1, IL3RA, NCAM1, CDKN1B, CDKN2A
8prolymphocytic leukemia10.6
9acute monocytic leukemia10.6
10hematopoietic stem cell transplantation10.6
11monocytic leukemia10.6
12cd4+/cd56+ hematodermic neoplasm10.6
13kaposi sarcoma10.4
14diffuse large b-cell lymphoma10.4
15t-cell large granular lymphocyte leukemia10.4
16t-cell leukemia10.4
17myelodysplastic syndrome10.4
18leprosy10.4
19post-transplant lymphoproliferative disease10.4
20cutaneous lupus erythematosus10.4
21macroglobulinemia10.4
22lupus erythematosus10.4
23melanoma10.4
24meningitis10.4
25purpura10.4
26sarcoma10.4
27dendritic cell tumor10.4
28endotheliitis10.4
29progressive multifocal leukoencephalopathy10.3CD4, SPIB
30nijmegen breakage syndrome10.3
31ataxia-telangiectasia10.3
32myocardial infarction10.3
33acute liver failure10.3
34anterior scleritis10.3
35panuveitis10.3
36scleritis10.3
37thyroiditis10.3
38vasculitis10.3
39ataxia10.3
40classic kaposi sarcoma10.3CD4
41pemphigus foliaceus10.3
42pemphigus10.3
43malignant histiocytosis10.3
44megaloblastic anemia10.3
45histiocytosis10.3
46large granular lymphocyte leukemia10.3
47tonsil cancer10.2CDKN1B, CDKN2A
48cervical adenocarcinoma10.2CDKN1B, CDKN2A
49cervical squamous cell carcinoma10.2CDKN1B, CDKN2A
50reticulosarcoma10.2CDKN1B, NPM1

Graphical network of the top 20 diseases related to Blastic Plasmacytoid Dendritic Cell:



Diseases related to blastic plasmacytoid dendritic cell

Symptoms for Blastic Plasmacytoid Dendritic Cell

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Drugs & Therapeutics for Blastic Plasmacytoid Dendritic Cell

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Drug clinical trials:

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Genetic Tests for Blastic Plasmacytoid Dendritic Cell

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Anatomical Context for Blastic Plasmacytoid Dendritic Cell

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MalaCards organs/tissues related to Blastic Plasmacytoid Dendritic Cell:

31
T cells, Myeloid, Skin, Bone marrow, Bone, Lung, Monocytes, B cells, Lymph node, Nk cells, Endothelial

Animal Models for Blastic Plasmacytoid Dendritic Cell or affiliated genes

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MGI Mouse Phenotypes related to Blastic Plasmacytoid Dendritic Cell:

35 (show all 11)
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020069.1TET2, MYD88, NPM1, CDKN2A, CDKN1B
2MP:00030129.0TET2, MYD88, CD4, CDKN2A, CDKN1B
3MP:00053708.8TET2, PECAM1, NPM1, CDKN2A, CDKN1B
4MP:00053918.8CDKN1B, CDKN2A, NPM1, NCAM1, MYD88
5MP:00053858.2TET2, PECAM1, MPO, MYD88, NPM1, CDKN1B
6MP:00053788.1TET2, MYD88, NCAM1, NPM1, CD4, CDKN2A
7MP:00053818.1SPIB, PECAM1, MYD88, CD4, CDKN2A, CDKN1B
8MP:00053977.3CDKN1B, TET2, SPIB, PECAM1, MPO, MYD88
9MP:00107687.3TET2, SPIB, MPO, MYD88, NCAM1, NPM1
10MP:00053877.3TET2, SPIB, PECAM1, MPO, MYD88, NPM1
11MP:00053847.2TET2, SPIB, PECAM1, MYD88, NCAM1, NPM1

Publications for Blastic Plasmacytoid Dendritic Cell

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Articles related to Blastic Plasmacytoid Dendritic Cell:

(show top 50)    (show all 132)
idTitleAuthorsYear
1
Blastic plasmacytoid dendritic cell neoplasm with leukemic manifestation and ETV6 gene rearrangement: A case report. (25780395)
2015
2
Blastic plasmacytoid dendritic cell neoplasm following WaldenstrAPm macroglobulinemia. (25765631)
2015
3
Rapid Progression of Blastic Plasmacytoid Dendritic Cell Neoplasm without Extracutaneous Manifestation. (25805691)
2015
4
Blastic plasmacytoid dendritic cell neoplasm: A case report. (25663917)
2015
5
Human BDCA2(+)CD123 (+)CD56 (+) dendritic cells (DCs) related to blastic plasmacytoid dendritic cell neoplasm represent a unique myeloid DC subset. (25779340)
2015
6
Blastic plasmacytoid dendritic cell neoplasm: skin and bone marrow infiltration of three cases and the review of the literature. (25825579)
2015
7
Two rare cases of blastic plasmacytoid dendritic cell neoplasm and a literature review. (24432892)
2014
8
Cutaneous lesions of a blastic plasmacytoid dendritic cell neoplasm. (24531095)
2014
9
Meningeal spread of blastic plasmacytoid dendritic cell neoplasm. (24483771)
2014
10
Blastic plasmacytoid dendritic cell neoplasm with extensive cutaneous and central nervous system involvement. (25568744)
2014
11
Blastic Plasmacytoid Dendritic Cell Neoplasm: Single-Center Experience with Two Cases in One Year. (25541663)
2014
12
Targeted ultra-deep sequencing reveals recurrent and mutually exclusive mutations of cancer genes in blastic plasmacytoid dendritic cell neoplasm. (25115387)
2014
13
Sustained remission of blastic plasmacytoid dendritic cell neoplasm after unrelated allogeneic stem cell transplantation-a single center experience. (25138222)
2014
14
Leukemic manifestation of blastic plasmacytoid dendritic cell neoplasm: laboratory approaches in 2 cases. (25325042)
2014
15
Blastic plasmacytoid dendritic cell neoplasm. (23582004)
2013
16
Stem cell transplantation can provide durable disease control in blastic plasmacytoid dendritic cell neoplasm: a retrospective study from the European Group for Blood and Marrow Transplantation. (23203822)
2013
17
L-asparaginase with methotrexate and dexamethasone is an effective treatment combination in blastic plasmacytoid dendritic cell neoplasm. (23937331)
2013
18
Blastic Plasmacytoid Dendritic Cell Neoplasm: Evolving Insights in an Aggressive Hematopoietic Malignancy With a Predilection of Skin Involvement. (24247574)
2013
19
Blastic plasmacytoid dendritic cell neoplasm in an elderly woman. (23629565)
2013
20
Lung involvement at initial presentation in blastic plasmacytoid dendritic cell neoplasm lacking cutaneous lesion. (22933234)
2013
21
Blastic plasmacytoid dendritic cell neoplasm: a report of four cases and review of the literature. (22455538)
2013
22
Reply to "A combination treatment approach and cord blood stem cell transplant for blastic plasmacytoid dendritic cell neoplasm" and to "A low-grade B-cell lymphoma with prolymphocytic/paraimmunoblastic proliferation and IRF4 rearrangement" dendritic cell leukemia. (23449597)
2013
23
SPIB, a novel immunohistochemical marker for human blastic plasmacytoid dendritic cell neoplasms: characterization of its expression in major hematolymphoid neoplasms. (23165482)
2013
24
Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study. (23065521)
2013
25
TET2 mutations, myelodysplastic features, and a distinct immunoprofile characterize blastic plasmacytoid dendritic cell neoplasm in the bone marrow. (23940084)
2013
26
Sustained complete remission in an elderly patient with a blastic plasmacytoid dendritic cell neoplasm following autologous peripheral blood stem cell transplantation. (23435845)
2013
27
Immunohistochemical analysis of CD123, CD56 and CD4 for the diagnosis of minimal bone marrow involvement by blastic plasmacytoid dendritic cell neoplasm. (23470050)
2013
28
Skin lesions in a patient with blastic plasmacytoid dendritic cell neoplasm. (24372537)
2013
29
Unusual Morphologic Presentations of Blastic Plasmacytoid Dendritic Cell Neoplasm: Report of Two Cases Misdiagnosed as Melanoma and Leprosy. (23515558)
2013
30
Characterization of CD56+ dendritic-like cells: a normal counterpart of blastic plasmacytoid dendritic cell neoplasm? (24312342)
2013
31
A combination treatment approach and cord blood stem cell transplant for blastic plasmacytoid dendritic cell neoplasm. (23300173)
2013
32
Childhood blastic plasmacytoid dendritic cell neoplasm treated with allogenic stem cell transplantation. (22300352)
2013
33
Leukemic manifestation of blastic plasmacytoid dendritic cell neoplasm lacking skin lesion : a borderline case between acute monocytic leukemia. (23037626)
2012
34
Intracytoplasmic detection of TCL1--but not ILT7-by flow cytometry is useful for blastic plasmacytoid dendritic cell leukemia diagnosis. (22674796)
2012
35
Loss of genomic DNA copy numbers in the p18, p16, p27 and RB loci in blastic plasmacytoid dendritic cell neoplasm. (22361914)
2012
36
Blastic plasmacytoid dendritic cell neoplasm in a child. (22583725)
2012
37
Cutaneous blastic plasmacytoid dendritic cell neoplasm occurring after spontaneous remission of acute myeloid leukemia: a case report and review of literature. (22476762)
2012
38
Congenital blastic plasmacytoid dendritic cell neoplasm. (21744480)
2012
39
Cutaneous accumulation of plasmacytoid dendritic cells associated with acute myeloid leukemia: a rare condition distinct from blastic plasmacytoid dendritic cell neoplasm. (21883371)
2011
40
An unusual case of cutaneous blastic plasmacytoid dendritic cell neoplasm with concomitant B-cell lymphoproliferative disorder. (20861710)
2011
41
Primary cutaneous blastic plasmacytoid dendritic cell neoplasm without extracutaneous manifestation: case report and review of the literature. (20573459)
2011
42
Blastic plasmacytoid dendritic cell neoplasm. (20940186)
2011
43
Bilateral conjunctival lesions in blastic plasmacytoid dendritic cell neoplasm. (21628861)
2011
44
Blastic plasmacytoid dendritic cell neoplasm presenting as a renal mass. (21939455)
2011
45
Blastic plasmacytoid dendritic cell neoplasm should be treated with acute leukemia type induction chemotherapy and allogeneic stem cell transplantation in first remission. (20697854)
2010
46
Blastic plasmacytoid dendritic cell neoplasm presenting as a subcutaneous mass in an 8-year-old boy. (20552185)
2010
47
Alterations of the cell-cycle inhibitors p27(KIP1) and p16(INK4a) are frequent in blastic plasmacytoid dendritic cell neoplasms. (19924135)
2010
48
A case of blastic plasmacytoid dendritic cell neoplasm initially mimicking cutaneous lupus erythematosus. (21253327)
2010
49
Blastic plasmacytoid dendritic cell neoplasm: is transplantation the treatment of choice? (19689477)
2010
50
Cutaneous blastic plasmacytoid dendritic cell neoplasm associated with anaemia and thrombocytopaenia. (19997703)
2009

Variations for Blastic Plasmacytoid Dendritic Cell

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Clinvar genetic disease variations for Blastic Plasmacytoid Dendritic Cell:

6
id Gene Variation Type Significance SNP ID Assembly Location
1NM_000051.3(ATM): c.7636_7644delTCTAGAATT (p.Arg2547_Ser2549del)deletionPathogenicGRCh38Chr 11, 108331885: 108331893

Expression for genes affiliated with Blastic Plasmacytoid Dendritic Cell

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Search GEO for disease gene expression data for Blastic Plasmacytoid Dendritic Cell.

Pathways for genes affiliated with Blastic Plasmacytoid Dendritic Cell

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Compounds for genes affiliated with Blastic Plasmacytoid Dendritic Cell

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Compounds related to Blastic Plasmacytoid Dendritic Cell according to GeneCards Suite gene sharing:

(show all 47)
idCompoundScoreTop Affiliating Genes
1[alcl]4310.2NPM1, CDKN1B
2hydralazine43 49 1212.1MPO, CDKN2A
3p000014310.0CDKN2A, CDKN1B
45 aminosalicylic acid4310.0MPO, PECAM1
5nickel43 2411.0CDKN2A, CD4
6cytarabine43 49 1211.9CDKN1B, CDKN2A, MPO
7daunorubicin43 49 1211.8MPO, NPM1, CDKN2A
8camptothecin43 59 1211.8NPM1, CDKN2A, CDKN1B
95fluorouracil439.8CDKN1B, MPO, CDKN2A
10rrna439.8NPM1, CDKN2A
11chloramphenicol43 2 49 1212.7CDKN2A, CD4, MPO
12methanol43 2410.7MPO, CDKN2A
13n acetylcysteine439.7MPO, CDKN2A, CDKN1B
14rantes439.7CD4, MYD88, PECAM1
15hematoxylin439.7CDKN2A, NCAM1, PECAM1
16agar439.6CDKN2A, CDKN1B, PECAM1
17butyrate439.6MPO, CDKN2A, CDKN1B
18fatty acid439.6NPM1, CDKN1B, CDKN2A, MYD88
19formaldehyde43 2410.5PECAM1, MPO, CD4, CDKN1B
20paclitaxel43 49 1211.5NPM1, CDKN2A, CDKN1B
21leucine439.5CDKN2A, NPM1, CD4, CDKN1B
22oligonucleotide439.5CDKN1B, MYD88, CD4, CDKN2A
23doxorubicin43 49 1211.5CDKN2A, CDKN1B, NPM1, NCAM1
24progesterone43 28 59 24 1213.5CD4, NCAM1, CDKN2A, CDKN1B
25actinomycin d439.4CDKN1B, CDKN2A, PECAM1, NPM1
26rapamycin439.4PECAM1, NPM1, CDKN1B
27h2o2439.4PECAM1, MPO, CDKN2A, CDKN1B
28cholesterol43 28 24 1212.4CD4, CDKN2A, CDKN1B, PECAM1
2912-o-tetradecanoylphorbol 13-acetate439.3CD4, NCAM1, IL3RA, CDKN1B
30oxygen43 2410.2MYD88, PECAM1, MPO, CDKN2A, CDKN1B
31alanine439.2CDKN2A, CD4, NPM1, NCAM1, CDKN1B
32cycloheximide439.1NPM1, CDKN2A, CD4, CDKN1B, PECAM1
33heparin43 28 24 1212.1CD4, NCAM1, MPO, PECAM1, CDKN1B
34vegf439.0PECAM1, NCAM1, CDKN1B, CDKN2A, MPO
35lipid439.0CDKN2A, CD4, PECAM1, NCAM1, MYD88
36phosphatidylinositol439.0IL3RA, MYD88, NCAM1, CD4, CDKN1B
37estrogen439.0CDKN1B, CDKN2A, NCAM1, PECAM1, NPM1
38histamine43 28 2411.0PECAM1, MPO, NCAM1
39cysteine439.0CDKN2A, CD4, PECAM1, NCAM1, NPM1
40nitric oxide43 24 1210.8PECAM1, CDKN1B, CDKN2A, MPO, MYD88, NCAM1
41atp43 289.8NCAM1, MYD88, PECAM1, NPM1, CDKN2A, CDKN1B
42paraffin438.7MPO, PECAM1, CDKN1B, CDKN2A, NPM1, NCAM1
43retinoic acid43 249.7MPO, CDKN1B, PECAM1, CDKN2A, NCAM1, NPM1
44calcium43 49 24 1211.6PECAM1, CD4, CDKN2A, NPM1, NCAM1, MPO
45serine438.0TCL1A, MYD88, NCAM1, CD4, PECAM1, MPO
46threonine437.3NPM1, CD4, CDKN1B, NCAM1, IL3RA, TCL1A
47tyrosine436.9CD4, NPM1, NCAM1, IL3RA, LILRA4, MYD88

GO Terms for genes affiliated with Blastic Plasmacytoid Dendritic Cell

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Biological processes related to Blastic Plasmacytoid Dendritic Cell according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1negative regulation of phosphorylationGO:00423269.8CDKN1B, CDKN2A
2negative regulation of cyclin-dependent protein serine/threonine kinase activityGO:00457369.5CDKN1B, CDKN2A
3negative regulation of apoptotic processGO:00430669.2CDKN1B, NPM1, MYD88, MPO

Molecular functions related to Blastic Plasmacytoid Dendritic Cell according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1cyclin-dependent protein serine/threonine kinase inhibitor activityGO:00048619.8CDKN1B, CDKN2A
2NF-kappaB bindingGO:00510599.5CDKN2A, NPM1
3protein kinase bindingGO:00199019.5CDKN2A, CD4, NPM1
4protein bindingGO:00055157.1TET2, PECAM1, MYD88, LILRA4, TCL1A, NPM1

Products for genes affiliated with Blastic Plasmacytoid Dendritic Cell

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Sources for Blastic Plasmacytoid Dendritic Cell

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet