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 20
Blastic Plasmacytoid Dendritic Cell Neoplasm 20 58 17 70
Blastic Nk-Cell Lymphoma 20 58 32
Lymphoblastoid Variant of Nk-Cell Lymphoma 20 58
Cd4+/cd56+ Hematodermic Neoplasm 20 58
Monomorphic Nk-Cell Lymphoma 20 58
Bpdcn 20 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 70 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 lymphoblastic and leukemia, acute myeloid. 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 Cell Differentiation Pathways and Lineage-specific Markers. The drugs Methotrexate and Dexamethasone have been mentioned in the context of this disorder. Affiliated tissues include myeloid, bone marrow 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 112)
# Related Disease Score Top Affiliating Genes
1 leukemia, acute lymphoblastic 30.6 NCAM1 MYC IL3RA
2 leukemia, acute myeloid 30.4 TET2 NCAM1 MYC IL3RA
3 myelodysplastic syndrome 30.2 TET2 MYC IL3RA
4 chronic myelomonocytic leukemia 30.2 TET2 MYC
5 merkel cell carcinoma 29.9 NCAM1 MYC
6 mature t-cell and nk-cell lymphoma 29.5 TET2 NCAM1 MYC
7 dendritic cell tumor 11.4
8 acute leukemia 10.8
9 myeloid leukemia 10.8
10 capillary leak syndrome 10.7
11 diphtheria 10.6
12 leukemia 10.6
13 thrombocytopenia 10.6
14 t-cell lymphoblastic leukemia/lymphoma 10.6
15 rare tumor 10.6
16 myeloid sarcoma 10.5
17 leukemia, t-cell, chronic 10.5
18 splenomegaly 10.5
19 hematologic cancer 10.5
20 lymphoma 10.4
21 sarcoma 10.4
22 pancytopenia 10.4
23 purpura 10.4
24 spindle cell sarcoma 10.4
25 acute monoblastic leukemia 10.4
26 leukemia, acute monocytic 10.3
27 lymphoma, hodgkin, classic 10.3
28 myelofibrosis 10.3
29 lymphoma, non-hodgkin, familial 10.3
30 hemophagocytic lymphohistiocytosis 10.3
31 cutaneous t cell lymphoma 10.3
32 lymphoproliferative syndrome 10.3
33 lymphoblastic lymphoma 10.3
34 b-lymphoblastic leukemia/lymphoma 10.3
35 monocytic leukemia 10.3
36 47,xyy 10.3
37 48,xyyy 10.3
38 posttransplant acute limbic encephalitis 10.3
39 kaposi sarcoma 10.2
40 chromosome 5q deletion syndrome 10.2
41 protoporphyria, erythropoietic, 1 10.2
42 retinoblastoma 10.2
43 triiodothyronine receptor auxiliary protein 10.2
44 anemia, autoimmune hemolytic 10.2
45 chondrosarcoma 10.2
46 leprosy 3 10.2
47 rheumatic fever-related antigen 10.2
48 xanthomatosis 10.2
49 tumor predisposition syndrome 10.2
50 graft-versus-host disease 10.2

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 67)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 126941
2
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
3
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
4
Idarubicin Approved Phase 2 58957-92-9 42890
5
leucovorin Approved Phase 2 58-05-9 6006
6
Fludarabine Approved Phase 1, Phase 2 21679-14-1, 75607-67-9 30751
7
Cyclophosphamide Approved, Investigational Phase 1, Phase 2 50-18-0, 6055-19-2 2907
8
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
9
Vincristine Approved, Investigational Phase 2 2068-78-2, 57-22-7 5978
10
Levoleucovorin Approved, Investigational Phase 2 68538-85-2 149436
11
Venetoclax Approved, Investigational Phase 2 1257044-40-8 49846579
12
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
13
Prednisolone acetate Approved, Vet_approved Phase 2 52-21-1
14
Mercaptopurine Approved Phase 2 50-44-2 667490
15
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
16
Cytarabine Approved, Investigational Phase 2 147-94-4 6253
17
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
18
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
19
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5
20
Daunorubicin Approved Phase 2 20830-81-3 30323
21
Ichthammol Approved Phase 2 8029-68-3
22
rituximab Approved Phase 2 174722-31-7 10201696
23
Azacitidine Approved, Investigational Phase 2 320-67-2 9444
24
Decitabine Approved, Investigational Phase 2 2353-33-5 451668
25
nivolumab Approved Phase 2 946414-94-4
26
Creatine Approved, Investigational, Nutraceutical Phase 1, Phase 2 57-00-1 586
27
Folic acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
28
Prednisolone hemisuccinate Experimental Phase 2 2920-86-7
29
Cortisone Experimental Phase 2 53-06-5 222786
30
asparaginase Phase 2
31 Antirheumatic Agents Phase 1, Phase 2
32 Immunosuppressive Agents Phase 1, Phase 2
33 Mitogens Phase 1, Phase 2
34 Alkylating Agents Phase 1, Phase 2
35 Immunologic Factors Phase 1, Phase 2
36 Gastrointestinal Agents Phase 2
37 Tubulin Modulators Phase 2
38 Folic Acid Antagonists Phase 2
39
protease inhibitors Phase 2
40
Liposomal doxorubicin Phase 2 31703
41 Vitamin B9 Phase 2
42 Hormones Phase 2
43 Methylprednisolone Acetate Phase 2
44 Antimitotic Agents Phase 2
45 Antiemetics Phase 2
46 Antibiotics, Antitubercular Phase 2
47 Immunoglobulins Phase 2
48 Folate Phase 2
49 Antineoplastic Agents, Hormonal Phase 2
50 Vitamin B Complex Phase 2

Interventional clinical trials:

(show all 16)
# Name Status NCT ID Phase Drugs
1 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
2 SL-401 in Patients With Acute Myeloid Leukemia (AML) and Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) Completed NCT02113982 Phase 1, Phase 2 SL-401
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 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 Recruiting NCT04109482 Phase 1, Phase 2 Fludarabine;Cyclophosphamide
5 Phase 2 Clinical Trial for Comprehensive Treatment Program for Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN): Tagraxofusp (SL-401) in Combination With HCVAD/Mini-CVD and VENETOCLAX Recruiting NCT04216524 Phase 2 Cyclophosphamide;Cytarabine;Dexamethasone;Doxorubicin;Mercaptopurine;Methotrexate;Methylprednisolone;Prednisone;Venetoclax;Vincristine
6 Tagraxofusp (SL-401) Therapy for Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) Patients Post-Autologous or Post-Allogeneic Hematopoietic Cell Transplantation Recruiting NCT04317781 Phase 2
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 Single-Arm, Open-Label Study of Nivolumab in Patients With Relapsed or Refractory Peripheral T-Cell Lymphoma (PTCL) Terminated NCT03075553 Phase 2
11 Phase 1 Study of Venetoclax, a BCL2 Antagonist, for Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) Recruiting NCT03485547 Phase 1 Venetoclax
12 Phase 1 Study of SL-401 in Combination With Azacitidine and Venetoclax in Relapsed/Refractory Acute Myeloid Leukemia (AML) and in Treatment-Naive Subjects With AML Not Eligible for Standard Induction and in Subjects With Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) or SL-401 in Combination With Azacitidine in Subjects With High-Risk Myelodysplastic Syndrome (MDS) Recruiting NCT03113643 Phase 1 Azacitidine;SL-401;Venetoclax
13 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
14 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
15 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
16 Blastic Plasmacytoid Dendritic Cell Neoplasm in Korean Population: A Multicenter Study Completed 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
Myeloid, Bone Marrow, T Cells, Bone, Skin, Endothelial, Lymph Node

Publications for Blastic Plasmacytoid Dendritic Cell

Articles related to Blastic Plasmacytoid Dendritic Cell:

(show top 50) (show all 463)
# Title Authors PMID Year
1
Diagnosis of rare subtypes of acute myeloid leukaemia and related neoplasms. 61
33676766 2021
2
Tagraxofusp Treatment: Implications for Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm. 61
33739343 2021
3
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 2021
4
Mutational analysis in different foci revealing the clonal evolution of blastic plasmacytoid dendritic cell neoplasm. 61
33174506 2021
5
A Very Rare Type of Leukemia in a Young Pediatric Patient Treated With Hematopoietic Stem Cell Transplantation: Blastic Plasmacytoid Dendritic Cell Neoplasm. 61
33235146 2021
6
Combination of Tagraxofusp and Azacitidine Is an Effective Option for Relapsed Blastic Plasmacytoid Dendritic Cell Neoplasm After Allogeneic Hematopoietic Stem-Cell Transplantation. 61
33795208 2021
7
Plasmacytoid dendritic cell expansion defines a distinct subset of RUNX1-mutated acute myeloid leukemia. 61
32871587 2021
8
Transcriptomic and genomic heterogeneity in blastic plasmacytoid dendritic cell neoplasms: from ontogeny to oncogenesis. 61
33687433 2021
9
Bromodomain and extra-terminal (BET) inhibitors in treating myeloid neoplasms. 61
33161793 2021
10
Dermoscopy of blastic plasmacytoid dendritic cell neoplasm in two patients. 61
33639013 2021
11
Cytologic features of blastic plasmacytoid dendritic cell neoplasm involving liver: A case report and literature review. 61
32852895 2021
12
Ionized Calcium Binding Adaptor Molecule 1 (IBA1): A Novel, Highly Sensitive, and Specific Marker for Histiocytic, Dendritic, and Monocytic Neoplasms. 61
33582751 2021
13
Blastic plasmacytoid dendritic cell neoplasm with genetic mutations in multiple epigenetic modifiers: a case report. 61
33530792 2021
14
Severe tumor lysis syndrome during the induction therapy for the treatment of blastic plasmacytoid dendritic cell neoplasm arising from myelodysplastic/myeloproliferative neoplasms. 61
33598264 2021
15
Blastic Plasmacytoid Dendritic Cell Neoplasm: Single Center Experience on a Rare Hematological Malignancy. 61
33707837 2021
16
Primary cutaneous blastic plasmacytoid dendritic cell neoplasm in a child: A challenging diagnosis and management. 61
33275310 2021
17
Blastic plasmacytoid dendritic cell neoplasm in a young patient. 61
33560790 2021
18
Acute leukemia as the initial presentation of blastic plasmacytoid dendritic cell neoplasm. 61
32880935 2021
19
Spontaneous Regression of Blastic Plasmacytoid Dendritic Cell Neoplasm Following Sepsis by Serratia marcescens: A Case Report and Literature Review. 61
33716255 2021
20
Pediatric blastic plasmacytoid dendritic cell neoplasm: report of four cases and review of literature. 61
33392975 2021
21
Specific cutaneous infiltrates in patients with haematological neoplasms: a retrospective study with 49 patients. 61
33403659 2021
22
MicroRNA profiling of blastic plasmacytoid dendritic cell neoplasm and myeloid sarcoma. 61
33405278 2020
23
Efficacy and safety of ruxolitinib for Covid-19 related acute respiratory distress syndrome in a patient with blastic plasmacytoid dendritic cell neoplasm (leukemic variant). 61
32902339 2020
24
Venetoclax monotherapy for cutaneous blastic plasmacytoid dendritic cell neoplasm. 61
32968828 2020
25
CD28/4-1BB CD123 CAR T cells in blastic plasmacytoid dendritic cell neoplasm. 61
32111969 2020
26
A Case Series of Blastic Plasmacytoid Dendritic Cell Neoplasia. 61
33457326 2020
27
Bortezomib, Lenalidomide, and Dexamethasone in Elderly Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm. 61
32917576 2020
28
Second primary malignancies in blastic plasmacytoid dendritic cell neoplasm: A national database study. 61
32278799 2020
29
Blastic Plasmacytoid Dendritic-Cell Neoplasm. 61
33252872 2020
30
Blastic plasmacytoid dendritic cell neoplasm with a history of cytopenia: A case report. 61
32374950 2020
31
Tagraxofusp and anti-CD123 in blastic plasmacytoid dendritic cell neoplasm: a new hope. 61
32955827 2020
32
Blastic plasmacytoid dendritic cell neoplasms: results of an international survey on 398 adult patients. 61
33027528 2020
33
Targeting the alpha subunit of IL-3 receptor (CD123) in patients with acute leukemia. 61
32692611 2020
34
CD123 as a Biomarker in Hematolymphoid Malignancies: Principles of Detection and Targeted Therapies. 61
33113953 2020
35
Blastic plasmacytoid dendritic cell neoplasm. A rare hematodermic malignancy. 61
31926915 2020
36
Apparent partial loss of CD123 expression in blastic plasmacytoid dendritic cell neoplasm after treatment with CD123-targeted therapy: A novel finding and possible diagnostic pitfall. 61
32770556 2020
37
Targeting CD123 in AML. 61
32862874 2020
38
Evidence for separate transformation to acute myeloid leukemia and blastic plasmacytoid dendritic cell neoplasm from a shared ancestral hematopoietic clone. 61
32366145 2020
39
CD123+CD4+CD56+ neoplasm: blastic plasmacytoid dendritic cell neoplasm or acute myeloid leukemia? 61
32970810 2020
40
Approval of tagraxofusp-erzs for blastic plasmacytoid dendritic cell neoplasm. 61
32841341 2020
41
Blastic Plasmacytoid Dendritic Cell Neoplasm: An Uncommon Entity to Consider in the Differential Diagnosis of Cutaneous Lymphoma. 61
32054364 2020
42
Chronic myelomonocytic leukemia and blastic plasmacytoid dendritic cell neoplasm. A case report and systematic review. 61
32830878 2020
43
Pseudo-Rosette-Forming Blastic Plasmacytoid Dendritic Cell Neoplasm 61
32292013 2020
44
Tagraxofusp followed by combined azacitidine and venetoclax in blastic plasmacytoid dendritic cell neoplasm: A case report and literature review. 61
32847479 2020
45
Juvenile Blastic Plasmacytoid Dendritic Cell Neoplasm. 61
32725252 2020
46
Blastic Plasmacytoid Dendritic Cell Neoplasm: Underlining the importance of an early diagnosis and the use of tagraxofusp therapy before wide dissemination. 61
32757292 2020
47
[Progress in diagnosis and treatment of pediatric blastic plasmacytoid dendritic-cell neoplasm]. 61
32842395 2020
48
Survival outcomes in blastic plasmacytoid dendritic cell neoplasm by first-line treatment and stem cell transplant. 61
32722779 2020
49
Clinical diversity and treatment approaches to blastic plasmacytoid dendritic cell neoplasm: a retrospective multicentre study. 61
31955469 2020
50
Tagraxofusp as treatment for patients with blastic plasmacytoid dendritic cell neoplasm. 61
32460559 2020

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 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
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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