BPDCN
MCID: BLS007
MIFTS: 38

Blastic Plasmacytoid Dendritic Cell (BPDCN)

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

Aliases & Classifications for Blastic Plasmacytoid Dendritic Cell

MalaCards integrated aliases for Blastic Plasmacytoid Dendritic Cell:

Name: Blastic Plasmacytoid Dendritic Cell 52
Blastic Plasmacytoid Dendritic Cell Neoplasm 52 58 17 71
Blastic Nk-Cell Lymphoma 52 58 32
Lymphoblastoid Variant of Nk-Cell Lymphoma 52 58
Cd4+/cd56+ Hematodermic Neoplasm 52 58
Monomorphic Nk-Cell Lymphoma 52 58
Bpdcn 52 58

Characteristics:

Orphanet epidemiological data:

58
cd4+/cd56+ hematodermic neoplasm
Inheritance: Not applicable; Prevalence: 1-5/10000 (Europe); Age of onset: Elderly; Age of death: elderly;

Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

ICD10 32 C86.4
ICD10 via Orphanet 33 C86.4
Orphanet 58 ORPHA86870
UMLS 71 C1301363

Summaries for Blastic Plasmacytoid Dendritic Cell

MalaCards based summary : Blastic Plasmacytoid Dendritic Cell, also known as blastic plasmacytoid dendritic cell neoplasm, is related to chronic myelomonocytic leukemia and myelodysplastic syndrome. An important gene associated with Blastic Plasmacytoid Dendritic Cell is IL3RA (Interleukin 3 Receptor Subunit Alpha), and among its related pathways/superpathways are Colorectal Cancer Metastasis and Embryonic and Induced Pluripotent Stem Cells and Lineage-specific Markers. The drugs Idarubicin and Fludarabine have been mentioned in the context of this disorder. Affiliated tissues include nk cells, myeloid and t cells, and related phenotype is Increased viability with TRAIL.

Related Diseases for Blastic Plasmacytoid Dendritic Cell

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

(show top 50) (show all 111)
# Related Disease Score Top Affiliating Genes
1 chronic myelomonocytic leukemia 30.1 TET2 MYC
2 myelodysplastic syndrome 29.9 TET2 MYC IL3RA
3 leukemia, acute myeloid 29.8 TET2 NCAM1 MYC IL3RA
4 merkel cell carcinoma 29.7 NCAM1 MYC
5 hematologic cancer 29.6 NCAM1 MYC IL3RA
6 dendritic cell tumor 11.4
7 myeloid leukemia 10.9
8 leukemia, acute lymphoblastic 10.8
9 acute leukemia 10.8
10 capillary leak syndrome 10.6
11 t-cell lymphoblastic leukemia/lymphoma 10.6
12 rare tumor 10.6
13 leukemia 10.5
14 thrombocytopenia 10.5
15 leukemia, t-cell, chronic 10.5
16 diphtheria 10.5
17 lymphoma 10.4
18 myeloid sarcoma 10.4
19 splenomegaly 10.4
20 purpura 10.4
21 acute monoblastic leukemia 10.4
22 leukemia, acute monocytic 10.3
23 hemophagocytic lymphohistiocytosis 10.3
24 cutaneous t cell lymphoma 10.3
25 lymphoproliferative syndrome 10.3
26 lymphoblastic lymphoma 10.3
27 pancytopenia 10.3
28 monocytic leukemia 10.3
29 48,xyyy 10.3
30 posttransplant acute limbic encephalitis 10.3
31 bone marrow necrosis 10.2
32 breast cancer 10.1
33 kaposi sarcoma 10.1
34 chromosome 5q deletion syndrome 10.1
35 protoporphyria, erythropoietic, 1 10.1
36 retinoblastoma 10.1
37 anemia, autoimmune hemolytic 10.1
38 chondrosarcoma 10.1
39 lymphoma, hodgkin, classic 10.1
40 leprosy 3 10.1
41 myelofibrosis 10.1
42 rheumatic fever-related antigen 10.1
43 xanthomatosis 10.1
44 lymphoma, non-hodgkin, familial 10.1
45 tumor predisposition syndrome 10.1
46 graft-versus-host disease 10.1
47 leukemia, acute lymphoblastic 3 10.1
48 cholangiocarcinoma 10.1
49 cutaneous lupus erythematosus 10.1
50 exanthem 10.1

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

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

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased viability with TRAIL GR00154-A 8.62 MYC TCF4

Drugs & Therapeutics for Blastic Plasmacytoid Dendritic Cell

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

(show top 50) (show all 68)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Idarubicin Approved Phase 2 58957-92-9 42890
2
Fludarabine Approved Phase 1, Phase 2 21679-14-1, 75607-67-9 30751
3
Decitabine Approved, Investigational Phase 1, Phase 2 2353-33-5 451668
4
Azacitidine Approved, Investigational Phase 2 320-67-2 9444
5
Venetoclax Approved, Investigational Phase 2 1257044-40-8 49846579
6
Methotrexate Approved Phase 2 59-05-2, 1959-05-2 126941
7
leucovorin Approved Phase 2 58-05-9 6006 143
8
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
9
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
10
Vincristine Approved, Investigational Phase 2 57-22-7, 2068-78-2 5978
11
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
12
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
13
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
14
Daunorubicin Approved Phase 2 20830-81-3 30323
15
Mercaptopurine Approved Phase 2 50-44-2 667490
16
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5
17
rituximab Approved Phase 2 174722-31-7 10201696
18
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
19
Ichthammol Approved Phase 2 8029-68-3
20
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
21
Cytarabine Approved, Experimental, Investigational Phase 2 147-94-4, 65-46-3 6253
22 Prednisolone acetate Approved, Vet_approved Phase 2 52-21-1
23
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
24
nivolumab Approved Phase 2 946414-94-4
25
Creatine Approved, Investigational, Nutraceutical Phase 1, Phase 2 57-00-1 586
26
Folic acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
27 Lorvotuzumab mertansine Investigational Phase 2 1008106-64-6
28
Prednisolone hemisuccinate Experimental Phase 2 2920-86-7
29
Cortisone Experimental Phase 2 53-06-5 222786
30 Maytansine Phase 2
31
asparaginase Phase 2
32 Mitogens Phase 1, Phase 2
33 Antimetabolites Phase 2
34 Immunoglobulins Phase 2
35 Hormones Phase 2
36 Anti-Infective Agents Phase 2
37 Antineoplastic Agents, Hormonal Phase 2
38 Vitamin B Complex Phase 2
39 Antibodies Phase 2
40
protease inhibitors Phase 2
41 Hormone Antagonists Phase 2
42 Methylprednisolone Acetate Phase 2
43 Folic Acid Antagonists Phase 2
44 Antiviral Agents Phase 2
45 Neuroprotective Agents Phase 2
46 Antibodies, Monoclonal Phase 2
47 Dermatologic Agents Phase 2
48 Tubulin Modulators Phase 2
49
Liposomal doxorubicin Phase 2 31703
50 Antiemetics Phase 2

Interventional clinical trials:

(show all 18)
# Name Status NCT ID Phase Drugs
1 SL-401 in Patients With Acute Myeloid Leukemia (AML) and Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) Completed NCT02113982 Phase 1, Phase 2 SL-401
2 An Open-label Phase II Study of Lorvotuzumab Mertansine (IMGN901) in CD56 Expressing Hematological Malignancies Completed NCT02420873 Phase 2 Lorvotuzumab Mertansine (IMGN901)
3 Therapy Targeting the Interleukin-3 Receptor (IL3R) for Patients With Relapsed or Refractory and Elderly or Poor-Risk Acute Myeloid Leukemia (AML) or High-Risk Myelodysplastic Syndrome With DTIL3 (IND# 11314): a Phase I/II Clinical Trial Completed NCT00397579 Phase 1, Phase 2 DT388IL3
4 Tagraxofusp (SL-401) Therapy for Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) Patients Post-Autologous or Post-Allogeneic Hematopoietic Cell Transplantation Recruiting NCT04317781 Phase 2
5 Combination Chemotherapy (Methotrexate, L-asparaginase, Idarubicin and Dexamethasone) in Patients With Newly Diagnosed Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) Recruiting NCT03599960 Phase 2 Chemotherapy
6 A Phase 1/2, Open Label, Multicenter Trial to Assess the Safety and Efficacy of MB-102 in Patients With Relapsed or Refractory Blastic Plasmacytoid Dendritic Cell Neoplasm, Acute Myeloid Leukemia, and High Risk Myelodysplastic Syndrome Recruiting NCT04109482 Phase 1, Phase 2 Decitabine;Fludarabine;Cyclophosphamide
7 A Phase 1/2, Multi-center, Open-label Study of IMGN632 Monotherapy Administered Intravenously in Patients With CD123-positive Acute Myeloid Leukemia and Other CD123-positive Hematologic Malignancies Recruiting NCT03386513 Phase 1, Phase 2 IMGN632
8 A Phase II Study of Venetoclax in Combination With 10-Day Decitabine in Newly Diagnosed Elderly or Relapsed/Refractory Acute Myeloid Leukemia and Relapsed High-Risk Myelodysplastic Syndrome and Blastic Plasmacytoid Dendritic Cell Neoplasm Recruiting NCT03404193 Phase 2 Decitabine;Venetoclax
9 Tagraxofusp in Patients With CD123+ or With Blastic Plasmacytoid Dendritic Cell Neoplasm Immunophenotype-like Acute Myeloid Leukemia Not yet recruiting NCT04342962 Phase 2 tagraxofusp
10 Phase 2 Clinical Trial for Comprehensive Treatment Program for Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN): SL-401 in Combination With HCVAD and VENETOCLAX Not yet recruiting NCT04216524 Phase 2 Cyclophosphamide;Cytarabine;Dexamethasone;Doxorubicin;Mercaptopurine;Methotrexate;Methylprednisolone;Prednisone;Venetoclax;Vincristine
11 A Phase 2 Study of Nonmyeloablative Conditioning With Transplantation of Partially Human Leukocyte Antigen (HLA)-Mismatched Bone Marrow and Post-transplant Cyclophosphamide for Patients With Hematologic Malignancies Suspended NCT02623439 Phase 2 Cyclophosphamide 50 mg/kg IV Days 3 and 4 post transplant
12 Phase 2 Single-Arm, Open-Label Study of Nivolumab in Patients With Relapsed or Refractory Peripheral T-Cell Lymphoma (PTCL) Terminated NCT03075553 Phase 2
13 Phase 1 Study of Venetoclax, a BCL2 Antagonist, for Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) Recruiting NCT03485547 Phase 1 Venetoclax
14 Phase I Study of Cellular Immunotherapy Using T Cells Lentivirally Transduced to Express a CD123-Specific, Hinge-Optimized, CD28-Costimulatory Chimeric Antigen Receptor and a Truncated EGFR for Patients With CD123+ Relapsed/Refractory Acute Myeloid Leukemia and Persistent/Recurrent Blastic Plasmacytoid Dendritic Cell Neoplasm Recruiting NCT02159495 Phase 1 cyclophosphamide;Fludarabine Phosphate
15 A Phase 1 Multiple Dose Study to Evaluate the Safety and Tolerability of XmAb®14045 in Patients With CD123-Expressing Hematologic Malignancies Recruiting NCT02730312 Phase 1
16 Phase 1, Open-label Dose-escalation and Dose-expansion Study to Evaluate the Safety, Expansion, Persistence and Clinical Activity of a Single Dose of UCART123 (Allogeneic Engineered T-cells Expressing Anti-CD123 Chimeric Antigen Receptor), Administered in Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) Terminated NCT03203369 Phase 1
17 Descriptive Study of the Efficacy of Treatments for Blastic Dendritic Cell Neoplasm (BPDCN): a Retrospective Study of Patients Diagnosed in France From 2000 to 2013 Completed NCT02859623
18 Blastic Plasmacytoid Dendritic Cell Neoplasm in Korean Population: A Multicenter Study Active, not recruiting NCT03974971

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:

40
Nk Cells, Myeloid, T Cells, Skin, Bone Marrow, Bone, Lung

Publications for Blastic Plasmacytoid Dendritic Cell

Articles related to Blastic Plasmacytoid Dendritic Cell:

(show top 50) (show all 419)
# Title Authors PMID Year
1
A Unique Case of a Pediatric Patient With Blastic Plasmacytoid Dendritic Cell Neoplasm, Guillain Barre Syndrome, and Hemophagocytic Lymphohistiocytosis. 61
30933025 2020
2
Blastic Plasmacytoid Dendritic Cell Neoplasm in Children. 61
32336423 2020
3
Chemotherapy Options for Blastic Plasmacytoid Dendritic Cell Neoplasm. 61
32336418 2020
4
Immunotherapies Targeting CD123 for Blastic Plasmacytoid Dendritic Cell Neoplasm. 61
32336421 2020
5
Tagraxofusp for Blastic Plasmacytoid Dendritic Cell Neoplasm. 61
32336420 2020
6
Molecular Features of Blastic Plasmacytoid Dendritic Cell Neoplasm: DNA Mutations and Epigenetics. 61
32336416 2020
7
Blastic plasmacytoid dendritic cell neoplasm. A study of three cases. 61
31350059 2020
8
Blastic Plasmacytoid Dendritic Cell Neoplasm: Clinical Presentation and Diagnosis. 61
32336414 2020
9
Blastic Plasmacytoid Dendritic Cell Neoplasm: The Dermatologist's Perspective. 61
32336415 2020
10
Novel Therapies for Blastic Plasmacytoid Dendritic Cell Neoplasm. 61
32336422 2020
11
Blastic Plasmacytoid Dendritic Cell Neoplasm: The European Perspective. 61
32336424 2020
12
Hematopoietic Cell Transplant for Blastic Plasmacytoid Dendritic Cell Neoplasm. 61
32336425 2020
13
Blastic Plasmacytoid Dendritic Cell Neoplasm in 2020 and Beyond. 61
32336426 2020
14
Cytogenetics of Blastic Plasmacytoid Dendritic Cell Neoplasm: Chromosomal Rearrangements and DNA Copy-Number Alterations. 61
32336417 2020
15
New perspectives in genetics and targeted therapy for blastic plasmacytoid dendritic cell neoplasm. 61
32234682 2020
16
Blastic plasmacytoid dendritic cell neoplasm: First retrospective study in the Czech Republic. 61
32064883 2020
17
Evidence for separate transformation to acute myeloid leukemia and blastic plasmacytoid dendritic cell neoplasm from a shared ancestral hematopoietic clone. 61
32366145 2020
18
Blastic Plasmacytoid Dendritic Cell Neoplasm: Case Report and Literature Overview. 61
32565565 2020
19
Blastic plasmacytoid dendritic cell neoplasm with a history of cytopenia: A case report. 61
32374950 2020
20
Tagraxofusp as treatment for patients with blastic plasmacytoid dendritic cell neoplasm. 61
32460559 2020
21
Dendritic Cell Leukemia: a Review. 61
32415624 2020
22
Whole-genome analysis uncovers recurrent IKZF1 inactivation and aberrant cell adhesion in blastic plasmacytoid dendritic cell neoplasm. 61
31846142 2020
23
Quick complete response achievement with venetoclax and azacitidine in a case of relapsed disseminated blastic plasmacytoid dendritic cell neoplasm. 61
32030448 2020
24
Immunophenotypic characterization of reactive and neoplastic plasmacytoid dendritic cells permits establishment of a 10-color flow cytometric panel for initial workup and residual disease evaluation of blastic plasmacytoid dendritic cell neoplasm. 61
32241840 2020
25
Second primary malignancies in blastic plasmacytoid dendritic cell neoplasm: a national database study. 61
32278799 2020
26
Acute leukemic variant of blastic plasmacytoid dendritic cell neoplasm at initial presentation. 61
32324873 2020
27
[Clinical and Pathological Characteristics of Blastic Plasmacytoid Dendritic Cell Neoplasm]. 61
32319389 2020
28
Pseudo-rosette forming Blastic plasmacytoid dendritic cell neoplasm. 61
32292013 2020
29
Blastic plasmacytoid dendritic cell neoplasm with t(2;8)(p12;q24). 61
31782605 2020
30
Cutaneous lymphoma in Japan, 2012-2017: A nationwide study. 61
32033869 2020
31
Comprehensive metagenomic analysis of blastic plasmacytoid dendritic cell neoplasm. 61
32182365 2020
32
Prognostic relevance of a blastic plasmacytoid dendritic cell neoplasm-like immunophenotype in cytogenetically normal acute myeloid leukemia patients. 61
32186422 2020
33
Blastic plasmacytoid dendritic cell neoplasm: diagnosis, manifestations, and treatment. 61
31972688 2020
34
Blastic Plasmacytoid Dendritic Cell Neoplasm: An Uncommon Entity to Consider in the Differential Diagnosis of Cutaneous Lymphoma. 61
32054364 2020
35
[Clinical Features of Patients with Blastic Plasmacytoid Dendritic Cell Neoplasm]. 61
32027294 2020
36
Blastic Plasmacytoid Dendritic Cell Neoplasia: A Single Center Experience 61
31752482 2020
37
FDA Approval Summary: Tagraxofusp-erzs For Treatment of Blastic Plasmacytoid Dendritic Cell Neoplasm. 61
31548341 2020
38
CD28/4-1BB CD123 CAR T cells in blastic plasmacytoid dendritic cell neoplasm. 61
32111969 2020
39
Role of tagraxofusp in treating blastic plasmacytoid dendritic cell neoplasm (BPDCN). 61
31801379 2020
40
Cutaneous blastic plasmacytoid dendritic cell neoplasm arising in the context of TET2 and ZRSR2 mutated clonal cytopenias of unknown significance, secondary to somatic copy number losses involving CDK2NA/2NB and MTAP. 61
31705546 2020
41
Case Report of Anti-CD123 Chimeric Antigen Receptor T-Cell Therapy Followed by Radiotherapy for a Recurrence of Blastic Plasmacytoid Dendritic Cell Neoplasm After Allogeneic Hematopoietic Stem Cell Transplantation. 61
32368098 2020
42
Clinical diversity and treatment approaches to blastic plasmacytoid dendritic cell neoplasm: a retrospective multicentre study. 61
31955469 2020
43
[Enhancer hijacking via disease-specific chromosomal translocation in blastic plasmacytoid dendritic cell neoplasm]. 61
32023603 2020
44
Cytogenetic Findings in a Case of Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN). 61
32161188 2020
45
Myeloid Neoplasms with Elevated Plasmacytoid Dendritic Cell Differentiation Reflect the Maturation Process of Dendritic Cells. 61
31876105 2020
46
Flow Cytometry Identifies a Spectrum of Maturation in Myeloid Neoplasms Having Plasmacytoid Dendritic Cell Differentiation. 61
30614203 2020
47
Features of non-activation dendritic state and immune deficiency in blastic plasmacytoid dendritic cell neoplasm (BPDCN). 61
31811114 2019
48
How should we diagnose and treat blastic plasmacytoid dendritic cell neoplasm patients? 61
31869411 2019
49
Tagraxofusp, a novel CD123-directed cytotoxin to treat blastic plasmacytoid dendritic cell neoplasm. 61
31942876 2019
50
Updates in Novel Therapies for Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN). 61
31853773 2019

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

GO Terms for Blastic Plasmacytoid Dendritic Cell

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

# Name GO ID Score Top Affiliating Genes
1 DNA-templated transcription, initiation GO:0006352 8.96 TCF4 MYC
2 MAPK cascade GO:0000165 8.8 NCAM1 MYC IL3RA

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

# Name GO ID Score Top Affiliating Genes
1 E-box binding GO:0070888 8.62 TCF4 MYC

Sources for Blastic Plasmacytoid Dendritic Cell

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
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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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