BPDCN
MCID: BLS007
MIFTS: 37

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 53
Blastic Plasmacytoid Dendritic Cell Neoplasm 53 59 72
Blastic Nk-Cell Lymphoma 53 59 33
Lymphoblastoid Variant of Nk-Cell Lymphoma 53 59
Cd4+/cd56+ Hematodermic Neoplasm 53 59
Monomorphic Nk-Cell Lymphoma 53 59
Bpdcn 53 59

Characteristics:

Orphanet epidemiological data:

59
cd4+/cd56+ hematodermic neoplasm
Inheritance: Not applicable; Prevalence: 1-5/10000 (Europe); Age of onset: Elderly; Age of death: elderly;

Classifications:



External Ids:

ICD10 33 C86.4
ICD10 via Orphanet 34 C86.4
Orphanet 59 ORPHA86870
UMLS 72 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 leukemia, acute myeloid and hematologic cancer. An important gene associated with Blastic Plasmacytoid Dendritic Cell is MYC (MYC Proto-Oncogene, BHLH Transcription Factor), and among its related pathways/superpathways are Cell adhesion molecules (CAMs) and Embryonic and Induced Pluripotent Stem Cell Differentiation Pathways and Lineage-specific Markers. The drugs Trastuzumab and Adenosine have been mentioned in the context of this disorder. Affiliated tissues include nk cells, myeloid and skin.

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 99)
# Related Disease Score Top Affiliating Genes
1 leukemia, acute myeloid 30.5 TET2 NCAM1 MYC
2 hematologic cancer 29.7 TET2 NCAM1 MYC
3 dendritic cell tumor 11.4
4 myeloid leukemia 10.8
5 lymphocytic leukemia 10.8
6 leukemia, acute lymphoblastic 10.8
7 acute leukemia 10.7
8 hematopoietic stem cell transplantation 10.7
9 t-cell leukemia 10.6
10 rare tumor 10.6
11 leukemia, t-cell, chronic 10.5
12 myelodysplastic syndrome 10.5
13 leukemia 10.5
14 chronic myelomonocytic leukemia 10.4
15 thrombocytopenia 10.4
16 myeloid sarcoma 10.4
17 splenomegaly 10.4
18 lymphoma 10.4
19 acute monoblastic leukemia 10.4
20 leukemia, acute monocytic 10.3
21 hemophagocytic lymphohistiocytosis 10.3
22 lymphoproliferative syndrome 10.3
23 diphtheria 10.3
24 pancytopenia 10.3
25 purpura 10.3
26 monocytic leukemia 10.3
27 48,xyyy 10.3
28 posttransplant acute limbic encephalitis 10.3
29 felty syndrome 10.2
30 bone marrow necrosis 10.2
31 breast cancer 10.1
32 chromosome 5q deletion syndrome 10.1
33 neurofibromatosis, type iv, of riccardi 10.1
34 protoporphyria, erythropoietic, 1 10.1
35 retinoblastoma 10.1
36 anemia, autoimmune hemolytic 10.1
37 chondrosarcoma 10.1
38 lymphoma, hodgkin, classic 10.1
39 leprosy 3 10.1
40 myelofibrosis 10.1
41 rheumatic fever-related antigen 10.1
42 xanthomatosis 10.1
43 lymphoma, non-hodgkin, familial 10.1
44 tumor predisposition syndrome 10.1
45 graft-versus-host disease 10.1
46 leukemia, acute lymphoblastic 3 10.1
47 cholangiocarcinoma 10.1
48 cutaneous lupus erythematosus 10.1
49 exanthem 10.1
50 cutaneous t cell lymphoma 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

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 54)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Trastuzumab Approved, Investigational Phase 2 180288-69-1 9903
2
Adenosine Approved, Investigational Phase 2 58-61-7 60961
3
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
4
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
5
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 126941
6
leucovorin Approved Phase 2 58-05-9 143 6006
7
Idarubicin Approved Phase 2 58957-92-9 42890
8
nivolumab Approved Phase 2 946414-94-4
9
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
10
Folic acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
11 Lorvotuzumab mertansine Investigational Phase 2 1008106-64-6
12 Antineoplastic Agents, Phytogenic Phase 2
13 Tubulin Modulators Phase 2
14 Maytansine Phase 2
15 Ado-trastuzumab emtansine Phase 2
16 Antimitotic Agents Phase 2
17 Gastrointestinal Agents Phase 2
18 Anti-Inflammatory Agents Phase 2
19 Antiemetics Phase 2
20 Folic Acid Antagonists Phase 2
21 Hormones Phase 2
22 glucocorticoids Phase 2
23 Nucleic Acid Synthesis Inhibitors Phase 2
24 Vitamin B9 Phase 2
25 Antineoplastic Agents, Hormonal Phase 2
26 Folate Phase 2
27 Anti-Bacterial Agents Phase 2
28 Topoisomerase Inhibitors Phase 2
29 Vitamin B Complex Phase 2
30 Hormone Antagonists Phase 2
31 Antibiotics, Antitubercular Phase 2
32 Dermatologic Agents Phase 2
33 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2
34 Peripheral Nervous System Agents Phase 2
35 Antimetabolites, Antineoplastic Phase 2
36 Antimetabolites Phase 2
37 Autonomic Agents Phase 2
38 BB 1101 Phase 2
39
asparaginase Phase 2
40 Antineoplastic Agents, Immunological Phase 2
41 Immunosuppressive Agents Phase 2
42 Immunologic Factors Phase 2
43 Antirheumatic Agents Phase 2
44 Alkylating Agents Phase 2
45 Antineoplastic Agents, Alkylating Phase 2
46
Venetoclax Approved, Investigational Phase 1 1257044-40-8 49846579
47
Fludarabine Approved Phase 1 21679-14-1, 75607-67-9 30751
48
Vidarabine Approved, Investigational Phase 1 24356-66-9 21704 32326
49 Anti-Infective Agents Phase 1
50 Vidarabine Phosphate Phase 1

Interventional clinical trials:

(show all 13)
# Name Status NCT ID Phase Drugs
1 An Open-label Phase II Study of Lorvotuzumab Mertansine (IMGN901) in CD56 Expressing Hematological Malignancies Completed NCT02420873 Phase 2 Lorvotuzumab Mertansine (IMGN901)
2 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
3 Combination Chemotherapy (Methotrexate, L-asparaginase, Idarubicin and Dexamethasone) in Patients With Newly Diagnosed Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) Recruiting NCT03599960 Phase 2 Chemotherapy
4 Phase 2 Single-Arm, Open-Label Study of Nivolumab in Patients With Relapsed or Refractory Peripheral T-Cell Lymphoma (PTCL) Recruiting NCT03075553 Phase 2
5 SL-401 in Patients With Acute Myeloid Leukemia (AML) and Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) Active, not recruiting NCT02113982 Phase 1, Phase 2 SL-401
6 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
7 Phase 1 Study of Venetoclax, a BCL2 Antagonist, for Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) Recruiting NCT03485547 Phase 1 Venetoclax
8 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
9 A Phase 1, Multi-center, Open-label Study of IMGN632 Administered Intravenously in Patients With Relapsed/Refractory CD123-positive Acute Myeloid Leukemia and Other CD123-positive Hematologic Malignancies Recruiting NCT03386513 Phase 1 IMGN632
10 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
11 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
12 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
13 Blastic Plasmacytoid Dendritic Cell Neoplasm in Korean Population: A Multicenter Study Active, not recruiting NCT03974971

Search NIH Clinical Center for Blastic Plasmacytoid Dendritic Cell

Inferred drug relations via UMLS 72 / NDF-RT 51 :


Tagraxofusp

Genetic Tests for Blastic Plasmacytoid Dendritic Cell

Anatomical Context for Blastic Plasmacytoid Dendritic Cell

MalaCards organs/tissues related to Blastic Plasmacytoid Dendritic Cell:

41
Nk Cells, Myeloid, Skin, T Cells, Bone, Bone Marrow, Lung

Publications for Blastic Plasmacytoid Dendritic Cell

Articles related to Blastic Plasmacytoid Dendritic Cell:

(show top 50) (show all 357)
# Title Authors PMID Year
1
Blastic plasmacytoid dendritic cell neoplasm with unusual lymphoid features and macrovacuoles. 38
30955062 2019
2
Venetoclax to treat relapsed blastic plasmacytoid dendritic cell neoplasm: A case-report and review of literature. 38
31415942 2019
3
Blastic Plasmacytoid Dendritic Cell Neoplasm with Leukemic Component 38
30819627 2019
4
Blastic plasmacytoid dendritic cell neoplasm. A study of three cases. 38
31350059 2019
5
Germline missense NF1 mutation in an elderly patient with a blastic plasmacytoid dendritic cell neoplasm. 38
30977107 2019
6
Blastic plasmacytoid dendritic cell neoplasm in the background of myeloproliferative disorder and chronic lymphocytic leukaemia. 38
31308179 2019
7
High ORRs Seen with Tagraxofusp-erzs in BPDCN. 38
31126990 2019
8
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) in leukaemic phase without skin lesions: a diagnostic and management challenge. 38
31003841 2019
9
Dual Expression of TCF4 and CD123 is Highly Sensitive and Specific For Blastic Plasmacytoid Dendritic Cell Neoplasm. 38
31261288 2019
10
Blastic Plasmacytoid Dendritic Cell Neoplasm-Current Insights. 38
31281107 2019
11
Clinical efficacy of bortezomib and lenalidomide in blastic plasmacytoid dendritic cell neoplasm. 38
30694364 2019
12
Multicenter Analysis of Outcomes in Blastic Plasmacytoid Dendritic Cell Neoplasm Offers a Pre-Targeted Therapy Benchmark. 38
31243042 2019
13
Blastic Plasmacytoid Dendritic Cell Neoplasm: First Case Report From Rwanda and Review of the Literature. 38
31246550 2019
14
PD1/PD-L1 Expression in Blastic Plasmacytoid Dendritic Cell Neoplasm. 38
31109153 2019
15
Chronic myelomonocytic leukaemia followed by blastic plasmacytoid dendritic cell neoplasm. 38
30784042 2019
16
Spinal Cord Toxicity from Intrathecal Chemotherapy: A Case with Clinicopathologic Correlation. 38
31128312 2019
17
Lineage-specific RUNX2 super-enhancer activates MYC and promotes the development of blastic plasmacytoid dendritic cell neoplasm. 38
30971697 2019
18
Tagraxofusp: First Global Approval. 38
30859413 2019
19
Tagraxofusp in Blastic Plasmacytoid Dendritic-Cell Neoplasm. 38
31018069 2019
20
Treatment advances in blastic plasmacytoid dendritic cell neoplasm. 38
31188810 2019
21
Blastic Plasmacytoid Dendritic Cell Neoplasm: A Case Report. 38
30734052 2019
22
Blastic Plasmacytoid Dendritic Cell Neoplasm: State of the Art and Prospects. 38
31035408 2019
23
Blastic plasmacytoid dendritic cell neoplasm: genomics mark epigenetic dysregulation as a primary therapeutic target. 38
30381297 2019
24
Expounding on the essence of epigenetic and genetic abnormalities in blastic plasmacytoid dendritic cell neoplasms. 38
30930334 2019
25
Daratumumab for treatment of blastic plasmacytoid dendritic cell neoplasm A single-case report. 38
30975908 2019
26
Blastic plasmacytoid dendritic cell neoplasm with response to pralatrexate. 38
30652209 2019
27
A Unique Case of a Pediatric Patient With Blastic Plasmacytoid Dendritic Cell Neoplasm, Guillain Barre Syndrome, and Hemophagocytic Lymphohistiocytosis. 38
30933025 2019
28
Benefit of electron beam therapy in combination with medium-dose systemic corticosteroid as a palliative treatment for elderly patients with blastic plasmacytoid dendritic cell neoplasm: case report and review of the literature. 38
29876950 2019
29
Blastic Plasmacytoid Dendritic Cell Neoplasm. 38
30715612 2019
30
Blastic plasmacytoid dendritic cell neoplasm: an early presentation. 38
30865410 2019
31
More on Blastic Plasmacytoid Dendritic-Cell Neoplasms. 38
30763185 2019
32
More on Blastic Plasmacytoid Dendritic-Cell Neoplasms. 38
30785714 2019
33
More on Blastic Plasmacytoid Dendritic-Cell Neoplasms. 38
30785715 2019
34
A Case of Blastic Plasmacytoid Dendritic Cell Neoplasm with Unusual Presentation 38
30040070 2019
35
Blastic plasmacytoid dendritic cell neoplasm with unusual extracutaneous manifestation: Two case reports and literature review. 38
30732162 2019
36
Blastic plasmacytoid dendritic cell neoplasm with immunoblastoid morphology and MYC rearrangement and overexpression. 38
30482401 2019
37
Blastic Plasmacytoid Dendritic Cell Neoplasm; A Report of Three Cases. 38
30666080 2019
38
Clinical Activity and Tolerability of SL-401 (Tagraxofusp): Recombinant Diphtheria Toxin and Interleukin-3 in Hematologic Malignancies. 38
30621282 2019
39
Successful outcome of second allogeneic bone marrow transplantation for blastic plasmacytoid dendritic cell neoplasm with MYC locus rearrangement. 38
31061790 2019
40
IFN-γ and TNF-α aggravate endothelial damage caused by CD123-targeted CAR T cell. 38
31417286 2019
41
Flow Cytometry Identifies a Spectrum of Maturation in Myeloid Neoplasms Having Plasmacytoid Dendritic Cell Differentiation. 38
30614203 2019
42
Atraumatic splenic ruptures triggered both remission and death in a single case of blastic plasmacytoid dendritic cell neoplasm. 38
30918143 2019
43
Recurrent 8q24 rearrangement in blastic plasmacytoid dendritic cell neoplasm: association with immunoblastoid cytomorphology, MYC expression, and drug response. 38
29795241 2018
44
Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) on Social Media: #BPDCN-Increasing Exposure over Two Years Since Inception of a Disease-Specific Twitter Community. 38
30338458 2018
45
Putative new childhood leukemia cancer predisposition syndrome caused by germline bi-allelic missense mutations in DDX41. 38
30144193 2018
46
Blastic Plasmacytoid Dendritic Cell Neoplasm. 38
30350260 2018
47
An unexpected diagnosis: leukaemic presentation of blastic plasmacytoid dendritic cell neoplasm with massive splenomegaly. 38
30274817 2018
48
The distinctive cytology and disease evolution of blastic plasmacytoid dendritic cell neoplasm. 38
30020546 2018
49
Incidental diagnosis of blastic plasmacytoid dendritic cell neoplasm in skin excision for basal cell carcinoma. 38
30094841 2018
50
Allogeneic Hematopoietic Cell Transplantation Is an Effective Treatment for Blastic Plasmacytoid Dendritic Cell Neoplasm in First Complete Remission: Systematic Review and Meta-analysis. 38
30145196 2018

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 positive regulation of I-kappaB kinase/NF-kappaB signaling GO:0043123 9.26 CD4 BRD4
2 response to estradiol GO:0032355 9.16 MYC CD4
3 positive regulation of transcription, DNA-templated GO:0045893 9.13 MYC CD4 BRD4
4 viral process GO:0016032 8.8 NCAM1 CD4 BRD4

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

# Name GO ID Score Top Affiliating Genes
1 virus receptor activity GO:0001618 8.62 NCAM1 CD4

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 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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