MCID: BLS007
MIFTS: 45

Blastic Plasmacytoid Dendritic Cell

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

Aliases & Classifications for Blastic Plasmacytoid Dendritic Cell

MalaCards integrated aliases for Blastic Plasmacytoid Dendritic Cell:

Name: Blastic Plasmacytoid Dendritic Cell 49
Blastic Plasmacytoid Dendritic Cell Neoplasm 49 55 69
Lymphoblastoid Variant of Nk-Cell Lymphoma 49 55
Cd4+/cd56+ Hematodermic Neoplasm 49 55
Monomorphic Nk-Cell Lymphoma 49 55
Blastic Nk-Cell Lymphoma 49 55
Bpdcn 49 55

Characteristics:

Orphanet epidemiological data:

55
cd4+/cd56+ hematodermic neoplasm
Inheritance: Not applicable; Prevalence: 1-5/10000 (Europe); Age of onset: Elderly; Age of death: elderly;

Classifications:



External Ids:

Orphanet 55 ORPHA86870
ICD10 via Orphanet 33 C86.4
ICD10 32 C86.4
UMLS 69 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 b-cell lymphomas. An important gene associated with Blastic Plasmacytoid Dendritic Cell is NCAM1 (Neural Cell Adhesion Molecule 1), and among its related pathways/superpathways are NF-kappaB Signaling and Cell adhesion molecules (CAMs). The drugs Adenosine and Trastuzumab have been mentioned in the context of this disorder. Affiliated tissues include nk cells, myeloid and t cells, and related phenotypes are cellular and hematopoietic system

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 all 42)
# Related Disease Score Top Affiliating Genes
1 leukemia, acute myeloid 30.3 CDKN2A IL3RA NCAM1 NPM1 TET2
2 b-cell lymphomas 30.1 CDKN1B CDKN2A TCL1A
3 leukemia 10.6
4 myeloid leukemia 10.4
5 tonsil cancer 10.3 CDKN1B CDKN2A
6 hematopoietic stem cell transplantation 10.2
7 small cell neuroendocrine carcinoma 10.2 CDKN2A NCAM1
8 primary cutaneous diffuse large b-cell lymphoma, leg type 10.2 CDKN2A MYD88
9 localized osteosarcoma 10.1 CDKN2A CLEC4C
10 cervical clear cell adenocarcinoma 10.1 CDKN2A NCAM1
11 leukemia, acute monocytic 10.1
12 lymphoma 10.1
13 monocytic leukemia 10.1
14 primary central nervous system lymphoma 10.1 CDKN2A MYD88
15 protoporphyria, erythropoietic 10.0
16 anemia, autoimmune hemolytic 10.0
17 leprosy 3 10.0
18 rheumatic fever-related antigen 10.0
19 xanthomatosis 10.0
20 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.0
21 myelodysplastic syndrome 10.0
22 autoimmune lymphoproliferative syndrome, type v 10.0
23 chops syndrome 10.0
24 acute leukemia 10.0
25 chronic myelomonocytic leukemia 10.0
26 hemolytic anemia 10.0
27 post-transplant lymphoproliferative disease 10.0
28 cutaneous lupus erythematosus 10.0
29 sarcoma 10.0
30 rheumatic fever 10.0
31 melanoma 10.0
32 purpura 10.0
33 polyradiculopathy 10.0
34 myeloid sarcoma 10.0
35 lupus erythematosus 10.0
36 macroglobulinemia 10.0
37 meningitis 10.0
38 dendritic cell tumor 10.0
39 pdgfrb-associated chronic eosinophilic leukemia 10.0
40 endotheliitis 10.0
41 hematologic cancer 10.0 CDKN1B IL3RA NPM1
42 primary effusion lymphoma 9.9 CDKN1B TCL1A

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:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 10.14 SPIB MYD88 NCAM1 CD4 NPM1 CDKN1B
2 hematopoietic system MP:0005397 10.06 IL3RA MYD88 TCF4 CD4 NPM1 CDKN1B
3 immune system MP:0005387 10 SPIB IL3RA MYD88 TCF4 CD4 NPM1
4 digestive/alimentary MP:0005381 9.91 SPIB MYD88 CD4 CDKN1B NR3C1 CDKN2A
5 mortality/aging MP:0010768 9.7 SPIB SUPT3H MYD88 NCAM1 CD4 NPM1
6 liver/biliary system MP:0005370 9.63 NPM1 CDKN1B NR3C1 PECAM1 CDKN2A TET2
7 no phenotypic analysis MP:0003012 9.17 CLEC4C MYD88 CD4 CDKN1B NR3C1 CDKN2A

Drugs & Therapeutics for Blastic Plasmacytoid Dendritic Cell

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

(show all 42)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Adenosine Approved, Investigational Phase 2 58-61-7 60961
2
Trastuzumab Approved, Investigational Phase 2 180288-69-1 9903
3
Tacrolimus Approved, Investigational Phase 2 104987-11-3 445643 439492
4
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 126941
5
Cyclophosphamide Approved, Investigational Phase 2,Phase 1 50-18-0, 6055-19-2 2907
6
Mycophenolic acid Approved Phase 2 24280-93-1 446541
7
Mycophenolate mofetil Approved, Investigational Phase 2 128794-94-5 5281078
8
Fludarabine Approved Phase 2,Phase 1 21679-14-1, 75607-67-9 30751
9
Vidarabine Approved, Investigational Phase 2,Phase 1 24356-66-9 21704 32326
10 Thiotepa Approved, Investigational Phase 2 52-24-4 5453
11
nivolumab Approved Phase 2 946414-94-4
12
leucovorin Approved, Nutraceutical Phase 2 58-05-9 143 6006
13
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
14 Ado-trastuzumab emtansine Phase 2
15 Antimitotic Agents Phase 2
16 Maytansine Phase 2
17 Antineoplastic Agents, Phytogenic Phase 2
18 Alkylating Agents Phase 2,Phase 1
19 Folic Acid Antagonists Phase 2
20 Nucleic Acid Synthesis Inhibitors Phase 2
21 Vidarabine Phosphate Phase 2,Phase 1
22 Dermatologic Agents Phase 2
23 Vitamin B Complex Phase 2
24 Antibodies, Monoclonal Phase 2
25 Anti-Bacterial Agents Phase 2
26 Anti-Infective Agents Phase 2,Phase 1
27 Immunoglobulins Phase 2,Phase 1
28 Immunosuppressive Agents Phase 2,Phase 1
29 Antimetabolites Phase 2,Phase 1
30 Antimetabolites, Antineoplastic Phase 2,Phase 1
31 Antibiotics, Antitubercular Phase 2
32 Antibodies Phase 2,Phase 1
33 Antirheumatic Agents Phase 2,Phase 1
34 Calcineurin Inhibitors Phase 2
35 Antitubercular Agents Phase 2
36 Antiviral Agents Phase 2,Phase 1
37 Folate Nutraceutical Phase 2
38 Vitamin B9 Nutraceutical Phase 2
39 Vaccines Phase 1
40
Miconazole Approved, Investigational, Vet_approved 22916-47-8 4189
41 Cyclosporins
42 Antifungal Agents

Interventional clinical trials:

(show all 13)

# Name Status NCT ID Phase Drugs
1 An Open-label Phase II Study of Lorvotuzumab Mertansine Completed NCT02420873 Phase 2 Lorvotuzumab Mertansine (IMGN901)
2 SL-401 in Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm or Acute Myeloid Leukemia Recruiting NCT02113982 Phase 1, Phase 2 SL-401
3 Nivolumab in Treating Patients With Relapsed or Refractory Peripheral T-cell Lymphoma Recruiting NCT03075553 Phase 2
4 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 Cyclophosphamide;Fludarabine Phosphate;Methotrexate;Mycophenolate Mofetil;Tacrolimus;Thiotepa
5 DT388IL3 Fusion Protein in Treating Patients With Acute Myeloid Leukemia or Myelodysplastic Syndromes Active, not recruiting NCT00397579 Phase 1, Phase 2 DT388IL3
6 MK2206 in Treating Younger Patients With Recurrent or Refractory Solid Tumors or Leukemia Completed NCT01231919 Phase 1 Akt inhibitor MK2206
7 Study to Evaluate the Safety and Clinical Activity of UCART123 in Patients With BPDCN Recruiting NCT03203369 Phase 1
8 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 cyclophosphamide;Fludarabine Phosphate
9 Study of IMGN632 in Patients With Relapse/Refractory AML, BPDCN, ALL, Other CD123+ Hem Malignancies Recruiting NCT03386513 Phase 1 IMGN632
10 PH 1 Study to Evaluate Safety and Tolerability of XmAb14045 in Patients With CD123-expressing Hematologic Malignancies Recruiting NCT02730312 Phase 1
11 Descriptive Study of the Efficacy of Treatments for Blastic Dendritic Cell Neoplasm (BPDCN) Completed NCT02859623
12 Salvia Hispanica Seed in Reducing Risk of Disease Recurrence in Patients With Non-Hodgkin Lymphoma Recruiting NCT02652715
13 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 fludarabine phosphate;cyclosporine;mycophenolate mofetil

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:

38
Nk Cells, Myeloid, T Cells, Skin, Bone, Bone Marrow, Lung

Publications for Blastic Plasmacytoid Dendritic Cell

Articles related to Blastic Plasmacytoid Dendritic Cell:

(show top 50) (show all 218)
# Title Authors Year
1
Blastic plasmacytoid dendritic cell neoplasm with unusual morphology, MYC rearrangement and TET2 and DNMT3A mutations. ( 29411884 )
2018
2
Skin lesions serve as clues to relapse of pediatric blastic plasmacytoid dendritic cell neoplasm. ( 29436012 )
2018
3
Clonal evolution in the transition from cutaneous disease to acute leukaemia suggested by liquid biopsy in blastic plasmacytoid dendritic cell neoplasm. ( 29419438 )
2018
4
Blastic plasmacytoid dendritic cell neoplasm with centrocyte-like morphology clinically simulating a melanocytic nevus. ( 29293270 )
2018
5
A blastic plasmacytoid dendritic cell neoplasm-like phenotype identifies a subgroup of npm1-mutated acute myeloid leukemia patients with worse prognosis. ( 29080220 )
2018
6
Detection of Minimal Bone Marrow involvement of Blastic Plasmacytoid Dendritic Cell Neoplastic Cells - CD303 immunostaining as a diagnostic tool. ( 29415975 )
2018
7
8q24/MYC rearrangement is a recurrent cytogenetic abnormality in blastic plasmacytoid dendritic cell neoplasms. ( 29407586 )
2018
8
Exploring the effect of chidamide on blastic plasmacytoid dendritic cell neoplasm: a case report and literature review. ( 29379294 )
2018
9
Bendamustine for relapsed blastic plasmacytoid dendritic cell leukaemia. ( 28620927 )
2017
10
Bortezomib as a new therapeutic approach for blastic plasmacytoid dendritic cell neoplasm. ( 28798071 )
2017
11
Complete monocular vision loss due to optic nerve involvement of blastic plasmacytoid dendritic cell neoplasm. ( 29276838 )
2017
12
Blastic Plasmacytoid Dendritic Cell Neoplasm Evaluated by FDG PET/CT. ( 28394841 )
2017
13
Blastic plasmacytoid dendritic cell neoplasm following acquired erythropoietic protoporphyria. ( 28966462 )
2017
14
Blastic plasmacytoid dendritic cell neoplasm presenting as leukemia without cutaneous lesion. ( 29237977 )
2017
15
Analysis of First-Year Twitter Metrics of a Rare Disease Community for Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) on Social Media: #BPDCN. ( 29064025 )
2017
16
Clinical features and treatment outcomes of blastic plasmacytoid dendritic cell neoplasm: a single-center experience in Korea. ( 28870016 )
2017
17
Blastic plasmacytoid dendritic cell neoplasm in the CSF. ( 29043229 )
2017
18
Pediatric Blastic Plasmacytoid Dendritic Cell Neoplasm: A Systematic Literature Review. ( 28906324 )
2017
19
Blastic plasmacytoid dendritic cell neoplasm in children: A review of two cases. ( 28856005 )
2017
20
Clinicopathological analysis of 46 cases with CD4+ and/or CD56+ immature hematolymphoid malignancy: reappraisal of blastic plasmacytoid dendritic cell and -related neoplasms. ( 28795410 )
2017
21
Localized skin-limited blastic plasmacytoid dendritic cell neoplasm: A subset with possible durable remission without transplantation. ( 28752118 )
2017
22
CD4-Negative Variant of Cutaneous Blastic Plasmacytoid Dendritic Cell Neoplasm With a Novel PBRM1 Mutation in an 11-Year-Old Girl. ( 28419186 )
2017
23
Childhood Blastic Plasmacytoid Dendritic Cell Neoplasm Mimicking Acute Rheumatic Fever: Report of an Unusual Clinical Presentation and Review of Literature. ( 28859047 )
2017
24
Image Gallery: A case of blastic plasmacytoid dendritic cell neoplasm. ( 29052874 )
2017
25
BET Inhibitors Target Blastic Plasmacytoid Dendritic Cell Neoplasms. ( 27913389 )
2017
26
Flow cytometry to identify bone-marrow relapse in blastic plasmacytoid dendritic cell neoplasm: a case report. ( 28830609 )
2017
27
ARID1A mutation in blastic plasmacytoid dendritic cell neoplasm. ( 28751556 )
2017
28
Recurrent MYB rearrangement in blastic plasmacytoid dendritic cell neoplasm. ( 28344318 )
2017
29
Novel treatment of blastic plasmacytoid dendritic cell neoplasm: A case report. ( 29390581 )
2017
30
Blastic plasmacytoid dendritic cell neoplasm with t(11;19)(q23;p13.3);KMT2A(MLL) rearranged: a diagnostic challenge. ( 27774582 )
2017
31
Successful treatment with biweekly CHOP for bone marrow relapse of blastic plasmacytoid dendritic cell neoplasm. ( 28321093 )
2017
32
Haematopoietic cell transplantation for blastic plasmacytoid dendritic cell neoplasm: a North American multicentre collaborative study. ( 28980314 )
2017
33
Blastic Plasmacytoid Dendritic Cell Neoplasm Presenting as Erythematous Nodules with Gallbladder Involvement. ( 28761306 )
2017
34
Splenic Rupture Secondary to Blastic Plasmacytoid Dendritic Cell Neoplasm. ( 28963621 )
2017
35
Concomitant Occurrence of Blastic Plasmacytoid Dendritic Cell Neoplasm and Acute Myeloid Leukaemia after Lenalidomide Treatment for. ( 28879716 )
2017
36
Blastic plasmacytoid dendritic cell neoplasm: a pathological illustration of two clinical cases. ( 28762083 )
2017
37
A Case of Blastic Plasmacytoid Dendritic Cell Neoplasm Extensively Studied by Flow Cytometry and Immunohistochemistry. ( 28409040 )
2017
38
Blastic plasmacytoid dendritic cell leukemia in a black malian. ( 28656936 )
2017
39
Novel Pathways and Potential Therapeutic Strategies for Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN): CD123 and Beyond. ( 29064022 )
2017
40
Blastic Plasmacytoid Dendritic Cell Neoplasm with Pulmonary Involvement and Atypical Skin Lesion. ( 28635683 )
2017
41
Blastic Plasmacytoid Dendritic Cell Neoplasm Associated with Dystrophic Xanthomatosis after Chemotherapy. ( 27535402 )
2016
42
Blastic plasmacytoid dendritic cell neoplasm. ( 27166602 )
2016
43
A critical review of treatment modalities for blastic plasmacytoid dendritic cell neoplasm. ( 27823644 )
2016
44
Blastic plasmacytoid dendritic cell neoplasm associated with chronic myelomonocytic leukemia. ( 28092882 )
2016
45
Blastic plasmacytoid dendritic cell neoplasm: diagnostic criteria and therapeutical approaches. ( 27264021 )
2016
46
Blastic plasmacytoid dendritic cell neoplasm frequently shows occult central nervous system involvement at diagnosis and benefits from intrathecal therapy. ( 26840087 )
2016
47
Cytomorphological features of blastic plasmacytoid dendritic cell neoplasm on FNA and cerebrospinal fluid cytology: A review of 6 cases. ( 26618304 )
2016
48
Treatment of blastic plasmacytoid dendritic cell neoplasm. ( 27913457 )
2016
49
Blastic Plasmacytoid Dendritic Cell Neoplasm: From Origin ofA the Cell to Targeted Therapies. ( 27026248 )
2016
50
Blastic plasmacytoid dendritic cell neoplasm with transient response to pralatrexate. ( 28013360 )
2016

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:

# Super pathways Score Top Affiliating Genes
1 11.97 MYD88 NCAM1 PECAM1 SPIB
2 11.71 CD4 NCAM1 PECAM1
3 11.5 CD4 IL3RA NCAM1
4 11.18 CDKN1B CDKN2A TCF4
5 10.89 CD4 CLEC4C IL3RA NCAM1 SPIB
6 10.64 MYD88 NR3C1

GO Terms for Blastic Plasmacytoid Dendritic Cell

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

# Name GO ID Score Top Affiliating Genes
1 protein complex GO:0043234 9.02 CDKN1B MYD88 NPM1 NR3C1 TCL1A

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 transcription, DNA-templated GO:0045893 9.35 CD4 CDKN2A NPM1 SUPT3H TCF4
2 negative regulation of cyclin-dependent protein serine/threonine kinase activity GO:0045736 9.26 CDKN1B CDKN2A
3 positive regulation of transcription by RNA polymerase II GO:0045944 9.17 BRD4 CDKN2A NPM1 NR3C1 SPIB TCF4
4 negative regulation of phosphorylation GO:0042326 9.16 CDKN1B CDKN2A

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

# Name GO ID Score Top Affiliating Genes
1 protein kinase binding GO:0019901 9.1 CD4 CDKN1B CDKN2A NPM1 NR3C1 TCL1A
2 cyclin-dependent protein serine/threonine kinase inhibitor activity GO:0004861 8.96 CDKN1B CDKN2A

Sources for Blastic Plasmacytoid Dendritic Cell

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