BPDCN
MCID: BLS007
MIFTS: 41

Blastic Plasmacytoid Dendritic Cell (BPDCN) malady

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

Aliases & Classifications for Blastic Plasmacytoid Dendritic Cell

Aliases & Descriptions for Blastic Plasmacytoid Dendritic Cell:

Name: Blastic Plasmacytoid Dendritic Cell 50
Blastic Plasmacytoid Dendritic Cell Neoplasm 50 56 69
Lymphoblastoid Variant of Nk-Cell Lymphoma 50 56
Cd4+/cd56+ Hematodermic Neoplasm 50 56
Monomorphic Nk-Cell Lymphoma 50 56
Blastic Nk-Cell Lymphoma 50 56
Bpdcn 50 56

Characteristics:

Orphanet epidemiological data:

56
cd4+/cd56+ hematodermic neoplasm
Inheritance: Not applicable; Prevalence: 1-5/10000 (Europe); Age of onset: Elderly; Age of death: elderly;

Classifications:



External Ids:

Orphanet 56 ORPHA86870
ICD10 via Orphanet 34 C86.4
ICD10 33 C86.4

Summaries for Blastic Plasmacytoid Dendritic Cell

MalaCards based summary : Blastic Plasmacytoid Dendritic Cell, also known as blastic plasmacytoid dendritic cell neoplasm, is related to leukemia and myeloid leukemia. An important gene associated with Blastic Plasmacytoid Dendritic Cell is TET2 (Tet Methylcytosine Dioxygenase 2), and among its related pathways/superpathways are NF-kappaB Signaling and Cell adhesion molecules (CAMs). The drugs nivolumab and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include nk cells, myeloid and skin, and related phenotypes are cellular and hematopoietic system

Related Diseases for Blastic Plasmacytoid Dendritic Cell

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



Diseases related to Blastic Plasmacytoid Dendritic Cell

Symptoms & Phenotypes for Blastic Plasmacytoid Dendritic Cell

MGI Mouse Phenotypes related to Blastic Plasmacytoid Dendritic Cell:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 10.02 CD4 CDKN1B CDKN2A MYD88 NCAM1 NPM1
2 hematopoietic system MP:0005397 9.91 PECAM1 SPIB TET2 CD4 CDKN1B CDKN2A
3 immune system MP:0005387 9.81 CD4 CDKN1B CDKN2A IL3RA MYD88 NPM1
4 digestive/alimentary MP:0005381 9.8 CDKN1B CDKN2A MYD88 PECAM1 SPIB CD4
5 neoplasm MP:0002006 9.35 CDKN1B CDKN2A MYD88 NPM1 TET2
6 no phenotypic analysis MP:0003012 9.1 CD4 CDKN1B CDKN2A CLEC4C MYD88 TET2

Drugs & Therapeutics for Blastic Plasmacytoid Dendritic Cell

Drugs for Blastic Plasmacytoid Dendritic Cell (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 40)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
nivolumab Approved Phase 2 946414-94-4
2
Cyclophosphamide Approved, Investigational Phase 2,Phase 1 50-18-0, 6055-19-2 2907
3
Fludarabine Approved Phase 2,Phase 1 21679-14-1, 75607-67-9 30751
4
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 126941
5
Mycophenolate mofetil Approved, Investigational Phase 2 128794-94-5 5281078
6
Mycophenolic acid Approved Phase 2 24280-93-1 446541
7
Tacrolimus Approved, Investigational Phase 2 104987-11-3 445643 439492
8
Thiotepa Approved Phase 2 52-24-4 5453
9
Vidarabine Approved Phase 2,Phase 1 24356-66-9 32326 21704
10
Trastuzumab Approved, Investigational Phase 2 180288-69-1 9903
11
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
12
leucovorin Approved, Nutraceutical Phase 2 58-05-9 54575, 6560146 143
13 Antibodies Phase 2
14 Antibodies, Monoclonal Phase 2
15 Immunoglobulins Phase 2
16 Alkylating Agents Phase 2,Phase 1
17 Anti-Bacterial Agents Phase 2
18 Antibiotics, Antitubercular Phase 2
19 Anti-Infective Agents Phase 2,Phase 1
20 Antimetabolites Phase 2,Phase 1
21 Antimetabolites, Antineoplastic Phase 2,Phase 1
22 Antineoplastic Agents, Alkylating Phase 2,Phase 1
23 Antirheumatic Agents Phase 2,Phase 1
24 Antiviral Agents Phase 2,Phase 1
25 Calcineurin Inhibitors Phase 2
26 Dermatologic Agents Phase 2
27 Folic Acid Antagonists Phase 2
28 Immunosuppressive Agents Phase 2,Phase 1
29 Nucleic Acid Synthesis Inhibitors Phase 2
30 Vidarabine Phosphate Phase 2,Phase 1
31 Vitamin B Complex Phase 2
32 Ado-trastuzumab emtansine Phase 2
33 Antimitotic Agents Phase 2
34 Antineoplastic Agents, Phytogenic Phase 2
35 Maytansine Phase 2
36 Folate Nutraceutical Phase 2
37 Vitamin B9 Nutraceutical Phase 2
38
Cyclosporine Approved, Investigational, Vet_approved 79217-60-0, 59865-13-3 5284373 6435893
39
Miconazole Approved, Investigational, Vet_approved 22916-47-8 4189
40 Antifungal Agents

Interventional clinical trials:

(show all 11)
id Name Status NCT ID Phase
1 SL-401 in Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm or Acute Myeloid Leukemia Recruiting NCT02113982 Phase 1, Phase 2
2 Nivolumab in Treating Patients With Relapsed or Refractory Peripheral T-cell Lymphoma Recruiting NCT03075553 Phase 2
3 Selective Depletion of CD45RA+ T Cells From Allogeneic Peripheral Blood Stem Cell Grafts From HLA-Matched Related and Unrelated Donors in Preventing GVHD Recruiting NCT02220985 Phase 2
4 An Open-label Phase II Study of Lorvotuzumab Mertansine Active, not recruiting NCT02420873 Phase 2
5 DT388IL3 Fusion Protein in Treating Patients With Acute Myeloid Leukemia or Myelodysplastic Syndromes Active, not recruiting NCT00397579 Phase 1, Phase 2
6 MK2206 in Treating Younger Patients With Recurrent or Refractory Solid Tumors or Leukemia Completed NCT01231919 Phase 1
7 Genetically Modified T-cell Immunotherapy in Treating Patients With Relapsed/Refractory Acute Myeloid Leukemia and Persistent/Recurrent Blastic Plasmacytoid Dendritic Cell Neoplasm Recruiting NCT02159495 Phase 1
8 PH 1 Study to Evaluate Safety and Tolerability of XmAb14045 in Patients With CD123-expressing Hematologic Malignancies Recruiting NCT02730312 Phase 1
9 Descriptive Study of the Efficacy of Treatments for Blastic Dendritic Cell Neoplasm (BPDCN) Completed NCT02859623
10 Salvia Hispanica Seed in Reducing Risk of Disease Recurrence in Patients With Non-Hodgkin Lymphoma Recruiting NCT02652715
11 Fludarabine Phosphate and Total-Body Radiation Followed by Donor Peripheral Blood Stem Cell Transplant and Immunosuppression in Treating Patients With Hematologic Malignancies Active, not recruiting NCT00014235

Search NIH Clinical Center for Blastic Plasmacytoid Dendritic Cell

Genetic Tests for Blastic Plasmacytoid Dendritic Cell

Anatomical Context for Blastic Plasmacytoid Dendritic Cell

MalaCards organs/tissues related to Blastic Plasmacytoid Dendritic Cell:

39
Nk Cells, Myeloid, Skin, Bone, Bone Marrow, Lung, Monocytes

Publications for Blastic Plasmacytoid Dendritic Cell

Articles related to Blastic Plasmacytoid Dendritic Cell:

(show top 50) (show all 180)
id Title Authors Year
1
Blastic plasmacytoid dendritic cell neoplasm with t(11;19)(q23;p13.3);KMT2A(MLL) rearranged: a diagnostic challenge. ( 27774582 )
2017
2
BET Inhibitors Target Blastic Plasmacytoid Dendritic Cell Neoplasms. ( 27913389 )
2017
3
Blastic plasmacytoid dendritic cell neoplasm with transient response to pralatrexate. ( 28013360 )
2016
4
Effective Treatment of a Childhood Blastic Plasmacytoid Dendritic Cell Neoplasm with a Cutaneous Tumor Alone by Stem Cell Transplantation with Reduced Intensity Conditioning. ( 27408349 )
2016
5
Blastic Plasmacytoid Dendritic Cell Neoplasm is Dependent on BCL-2 and Sensitive to Venetoclax. ( 27986708 )
2016
6
Blastic plasmacytoid dendritic cell neoplasm: diagnostic criteria and therapeutical approaches. ( 27264021 )
2016
7
Blastic Plasmacytoid Dendritic Cell Neoplasm Associated with Dystrophic Xanthomatosis after Chemotherapy. ( 27535402 )
2016
8
Acute Myeloid Leukemia With MLL Rearrangement and CD4+/CD56+ Expression can be Misdiagnosed as Blastic Plasmacytoid Dendritic Cell Neoplasm: Two Case Reports. ( 27374717 )
2016
9
A Druggable TCF4- and BRD4-Dependent Transcriptional Network Sustains Malignancy in Blastic Plasmacytoid Dendritic Cell Neoplasm. ( 27846392 )
2016
10
Treatment of blastic plasmacytoid dendritic cell neoplasm. ( 27913457 )
2016
11
A rare case of blastic plasmacytoid dendritic cell neoplasm with deletion 7q.31, in the setting of heavy pre-treatment with alkylating chemotherapy. ( 27559020 )
2016
12
Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: 10-Color flow cytometry diagnosis and HyperCVAD therapy. ( 26619305 )
2016
13
Blastic plasmacytoid dendritic cell neoplasm: A case report and literature review. ( 27347056 )
2016
14
Can reduced intensity conditioning regimen cure blastic plasmacytoid dendritic cell neoplasm? ( 27986653 )
2016
15
Azacitidine in the treatment of blastic plasmacytoid dendritic cell neoplasm: a report of 3 cases. ( 26984056 )
2016
16
A critical review of treatment modalities for blastic plasmacytoid dendritic cell neoplasm. ( 27823644 )
2016
17
Cytomorphological features of blastic plasmacytoid dendritic cell neoplasm on FNA and cerebrospinal fluid cytology: A review of 6 cases. ( 26618304 )
2016
18
Myeloid Cell Nuclear Differentiation Antigen (MNDA) Expression Distinguishes Extramedullary Presentations of Myeloid Leukemia From Blastic Plasmacytoid Dendritic Cell Neoplasm. ( 26796502 )
2016
19
Simultaneous deletion of 3'ETV6 and 5'EWSR1 genes in blastic plasmacytoid dendritic cell neoplasm: case report and literature review. ( 26925167 )
2016
20
Blastic plasmacytoid dendritic cell neoplasm accompanied by autoimmune hemolytic anemia achieving complete remission with hydroxyurea and steroids. ( 26936199 )
2016
21
Blastic plasmacytoid dendritic cell neoplasm (BPDCN): a rare entity. ( 26791132 )
2016
22
CT findings associated with blastic plasmacytoid dendritic cell neoplasm: a case report. ( 27504192 )
2016
23
Case report of blastic plasmacytoid dendritic cell neoplasm (BPDCN) a rare entity. ( 27773220 )
2016
24
Blastic plasmacytoid dendritic cell neoplasm frequently shows occult central nervous system involvement at diagnosis and benefits from intrathecal therapy. ( 26840087 )
2016
25
Social Media and Internet Resources for Patients with Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN). ( 27492117 )
2016
26
Blastic Plasmacytoid Dendritic Cell Neoplasm: From Origin ofA the Cell to Targeted Therapies. ( 27026248 )
2016
27
Blastic plasmacytoid dendritic cell neoplasm. ( 27166602 )
2016
28
Blastic plasmacytoid dendritic cell neoplasm associated with chronic myelomonocytic leukemia. ( 28092882 )
2016
29
Haploinsufficiency for NR3C1, the gene encoding the glucocorticoid receptor, in blastic plasmacytoid dendritic cell neoplasms. ( 27060168 )
2016
30
LXR agonist treatment of blastic plasmacytoid dendritic cell neoplasm restores cholesterol efflux and triggers apoptosis. ( 27702801 )
2016
31
Classification and clinical behavior of blastic plasmacytoid dendritic cell neoplasms according to their maturation-associated immunophenotypic profile. ( 26056082 )
2015
32
Blastic plasmacytoid dendritic cell neoplasm with leukemic manifestation and ETV6 gene rearrangement: A case report. ( 25780395 )
2015
33
Blastic plasmacytoid dendritic cell neoplasm: skin and bone marrow infiltration of three cases and the review of the literature. ( 25825579 )
2015
34
Blastic Plasmacytoid Dendritic Cell Neoplasm: Analysis of Clinicopathological Feature and Treatment Outcome of Seven Cases. ( 26719643 )
2015
35
Colon Involvement in Blastic Plasmacytoid Dendritic Cell Neoplasm. ( 26134209 )
2015
36
Long-term survival following autologous and allogeneic stem cell transplantation for blastic plasmacytoid dendritic cell neoplasm. ( 25918345 )
2015
37
Blastic Plasmacytoid Dendritic Cell Neoplasm in the Pediatric Population: A Case Series and Review of the Literature. ( 26588336 )
2015
38
Increased plasma d-2-hydroxyglutarate in isocitrate dehydrogenase 2-mutated blastic plasmacytoid dendritic cell neoplasm. ( 25481493 )
2015
39
Blastic plasmacytoid dendritic cell neoplasm: report of two pediatric cases. ( 25673598 )
2015
40
KMT2A (MLL)-MLLT1 rearrangement in blastic plasmacytoid dendritic cell neoplasm. ( 26164398 )
2015
41
BLASTIC PLASMACYTOID DENDRITIC CELL NEOPLASM --A RAPIDLY EVOLVING ENTITY. CASE REPORT. ( 26204640 )
2015
42
Blastic plasmacytoid dendritic cell neoplasm: two case reports. ( 26553703 )
2015
43
Radiation therapy for cutaneous blastic plasmacytoid dendritic cell neoplasm: a case report and review of the literature. ( 26221394 )
2015
44
Infiltrative plaques on the temple. Blastic plasmacytoid dendritic cell neoplasm (BPDCN). ( 25801079 )
2015
45
Identification of SUPT3H as a novel 8q24/MYC partner in blastic plasmacytoid dendritic cell neoplasm with t(6;8)(p21;q24) translocation. ( 25860292 )
2015
46
Strong periodic acid-schiff reaction in hypervacuolated blastic plasmacytoid dendritic cell neoplasm. ( 26467980 )
2015
47
Rapid Progression of Blastic Plasmacytoid Dendritic Cell Neoplasm without Extracutaneous Manifestation. ( 25805691 )
2015
48
Allogeneic Hematopoietic Stem Cell Transplantions in Blastic Plasmacytoid Dendritic Cell Neoplasm in first complete remission: an effective therapy for a rare disease. ( 26699933 )
2015
49
Blastic plasmacytoid dendritic cell neoplasm following WaldenstrAPm macroglobulinemia. ( 25765631 )
2015
50
Leukemic Polyradiculopathy Due to Blastic Plasmacytoid Dendritic Cell Neoplasm. ( 26099022 )
2015

Variations for Blastic Plasmacytoid Dendritic Cell

Expression for Blastic Plasmacytoid Dendritic Cell

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

Pathways for Blastic Plasmacytoid Dendritic Cell

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

id Super pathways Score Top Affiliating Genes
1 11.75 MYD88 NCAM1 PECAM1 SPIB
2 11.59 CD4 NCAM1 PECAM1
3 11.4 CD4 IL3RA NCAM1
4 10.9 CD4 CLEC4C IL3RA NCAM1 SPIB

GO Terms for Blastic Plasmacytoid Dendritic Cell

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

id Name GO ID Score Top Affiliating Genes
1 immune system process GO:0002376 9.46 CD4 CLEC4C LILRA4 MYD88
2 adaptive immune response GO:0002250 9.43 CD4 CLEC4C LILRA4
3 negative regulation of cyclin-dependent protein serine/threonine kinase activity GO:0045736 8.96 CDKN1B CDKN2A
4 negative regulation of phosphorylation GO:0042326 8.62 CDKN1B CDKN2A

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

id Name GO ID Score Top Affiliating Genes
1 protein kinase binding GO:0019901 9.26 CD4 CDKN1B CDKN2A NPM1
2 NF-kappaB binding GO:0051059 9.16 CDKN2A NPM1
3 cyclin-dependent protein serine/threonine kinase inhibitor activity GO:0004861 8.62 CDKN1B CDKN2A

Sources for Blastic Plasmacytoid Dendritic Cell

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