MCID: BLS007
MIFTS: 39

Blastic Plasmacytoid Dendritic Cell

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 54
Blastic Plasmacytoid Dendritic Cell Neoplasm 54 60 74
Lymphoblastoid Variant of Nk-Cell Lymphoma 54 60
Cd4+/cd56+ Hematodermic Neoplasm 54 60
Monomorphic Nk-Cell Lymphoma 54 60
Blastic Nk-Cell Lymphoma 54 60
Bpdcn 54 60

Characteristics:

Orphanet epidemiological data:

60
cd4+/cd56+ hematodermic neoplasm
Inheritance: Not applicable; Prevalence: 1-5/10000 (Europe); Age of onset: Elderly; Age of death: elderly;

Classifications:



External Ids:

ICD10 34 C86.4
ICD10 via Orphanet 35 C86.4
Orphanet 60 ORPHA86870
UMLS 74 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 leukemia, acute myeloid. An important gene associated with Blastic Plasmacytoid Dendritic Cell is MYC (MYC Proto-Oncogene, BHLH Transcription Factor), and among its related pathways/superpathways are Embryonic and Induced Pluripotent Stem Cell Differentiation Pathways and Lineage-specific Markers and Dendritic Cells Developmental Lineage Pathway. The drugs Adenosine and Trastuzumab have been mentioned in the context of this disorder. Affiliated tissues include nk cells, myeloid and t cells.

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 52)
# Related Disease Score Top Affiliating Genes
1 chronic myelomonocytic leukemia 30.4 DNMT3A TET2
2 leukemia, acute myeloid 29.9 DNMT3A MYC NCAM1 TET2
3 dendritic cell tumor 11.3
4 leukemia 10.8
5 myeloid leukemia 10.6
6 lymphoma 10.4
7 pdgfrb-associated chronic eosinophilic leukemia 10.4
8 leukemia, acute monocytic 10.3
9 retinoblastoma 10.3
10 myelodysplastic syndrome 10.3
11 acute leukemia 10.3
12 hematopoietic stem cell transplantation 10.3
13 monocytic leukemia 10.3
14 acute monoblastic leukemia 10.3
15 protoporphyria, erythropoietic, 1 10.1
16 anemia, autoimmune hemolytic 10.1
17 leprosy 3 10.1
18 rheumatic fever-related antigen 10.1
19 spondylocarpotarsal synostosis syndrome 10.1
20 xanthomatosis 10.1
21 basal cell carcinoma 1 10.1
22 hemolytic anemia 10.1
23 hemophagocytic lymphohistiocytosis 10.1
24 post-transplant lymphoproliferative disease 10.1
25 waldenstrom macroglobulinemia 10.1
26 cutaneous lupus erythematosus 10.1
27 lymphoproliferative syndrome 10.1
28 sarcoma 10.1
29 cauda equina syndrome 10.1
30 guillain-barre syndrome 10.1
31 rheumatic fever 10.1
32 melanoma 10.1
33 basal cell carcinoma 10.1
34 purpura 10.1
35 polyradiculopathy 10.1
36 mucormycosis 10.1
37 myeloid sarcoma 10.1
38 lupus erythematosus 10.1
39 macroglobulinemia 10.1
40 hansen's disease 10.1
41 splenomegaly 10.1
42 diphtheria 10.1
43 lymphatic system cancer 10.0 MYC NCAM1
44 mature b-cell neoplasm 10.0 MYC NCAM1
45 autonomic nervous system neoplasm 9.9 MYC NCAM1
46 peripheral nervous system neoplasm 9.9 MYC NCAM1
47 leukocyte disease 9.9 MYC NCAM1
48 peripheral t-cell lymphoma 9.9 NCAM1 TET2
49 small cell cancer of the lung 9.8 MYC NCAM1
50 juvenile myelomonocytic leukemia 9.8 DNMT3A TET2

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 65)
# 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
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
4
Idarubicin Approved Phase 2 58957-92-9 42890
5
leucovorin Approved Phase 2 58-05-9 143 6006
6
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 126941
7
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
8
nivolumab Approved Phase 2 946414-94-4
9
Fludarabine Approved Phase 2,Phase 1,Not Applicable 75607-67-9, 21679-14-1 30751
10
Mycophenolic acid Approved Phase 2,Not Applicable 24280-93-1 446541
11
Vidarabine Approved, Investigational Phase 2,Phase 1,Not Applicable 24356-66-9 21704 32326
12
Thiotepa Approved, Investigational Phase 2 52-24-4 5453
13
Tacrolimus Approved, Investigational Phase 2 104987-11-3 6473866 445643 439492
14
Cyclophosphamide Approved, Investigational Phase 2,Phase 1 50-18-0, 6055-19-2 2907
15
Busulfan Approved, Investigational Phase 2 55-98-1 2478
16
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
17 Lorvotuzumab mertansine Investigational Phase 2 1008106-64-6
18
Emodepside Investigational, Vet_approved Phase 2 155030-63-0
19 Antimitotic Agents Phase 2
20 Ado-trastuzumab emtansine Phase 2
21 Antineoplastic Agents, Immunological Phase 2
22 Maytansine Phase 2
23 Antineoplastic Agents, Phytogenic Phase 2
24 Hormones Phase 2
25 Hormone Antagonists Phase 2
26 Vitamin B9 Phase 2
27 Gastrointestinal Agents Phase 2
28 Antiemetics Phase 2
29 Folate Phase 2
30 Antineoplastic Agents, Hormonal Phase 2
31 Antirheumatic Agents Phase 2,Phase 1,Not Applicable
32 Antibiotics, Antitubercular Phase 2,Not Applicable
33 Peripheral Nervous System Agents Phase 2
34 Immunosuppressive Agents Phase 2,Phase 1,Not Applicable
35 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2
36 glucocorticoids Phase 2
37 Dermatologic Agents Phase 2,Not Applicable
38
asparaginase Phase 2
39 Folic Acid Antagonists Phase 2
40 Immunologic Factors Phase 2,Phase 1,Not Applicable
41 Anti-Bacterial Agents Phase 2,Not Applicable
42 Vitamin B Complex Phase 2
43 Antimetabolites, Antineoplastic Phase 2,Phase 1,Not Applicable
44 BB 1101 Phase 2
45 Anti-Inflammatory Agents Phase 2
46 Nucleic Acid Synthesis Inhibitors Phase 2
47 Topoisomerase Inhibitors Phase 2
48 Antimetabolites Phase 2,Phase 1,Not Applicable
49 Autonomic Agents Phase 2
50 Antineoplastic Agents, Alkylating Phase 2,Phase 1

Interventional clinical trials:

(show all 19)
# Name Status NCT ID Phase Drugs
1 DT388IL3 Fusion Protein in Treating Patients With Acute Myeloid Leukemia or Myelodysplastic Syndromes Completed NCT00397579 Phase 1, Phase 2 DT388IL3
2 An Open-label Phase II Study of Lorvotuzumab Mertansine Completed NCT02420873 Phase 2 Lorvotuzumab Mertansine (IMGN901)
3 Combination Chemotherapy in Patients With Newly Diagnosed BPDCN Recruiting NCT03599960 Phase 2 Chemotherapy
4 Nivolumab in Treating Patients With Relapsed or Refractory Peripheral T-cell Lymphoma Recruiting NCT03075553 Phase 2
5 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
6 SL-401 in Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm or Acute Myeloid Leukemia Active, not recruiting NCT02113982 Phase 1, Phase 2 SL-401
7 Flotetuzumab in Treating Patients With Recurrent or Refractory CD123 Positive Blood Cancer Not yet recruiting NCT03739606 Phase 2
8 Naive T Cell Depletion for Preventing Chronic Graft-versus-Host Disease in Children and Young Adults With Blood Cancers Undergoing Donor Stem Cell Transplant Not yet recruiting NCT03779854 Phase 2 Thiotepa;Fludarabine;Cyclophosphamide;Busulfan;Tacrolimus;Methotrexate
9 MK2206 in Treating Younger Patients With Recurrent or Refractory Solid Tumors or Leukemia Completed NCT01231919 Phase 1 Akt inhibitor MK2206
10 Study of Venetoclax, a BCL2 Antagonist, for Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) Recruiting NCT03485547 Phase 1 Venetoclax
11 Study to Evaluate the Safety and Clinical Activity of UCART123 in Patients With BPDCN Recruiting NCT03203369 Phase 1
12 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
13 Study of IMGN632 in Patients With Relapse/Refractory AML, BPDCN, ALL, Other CD123+ Hem Malignancies Recruiting NCT03386513 Phase 1 IMGN632
14 PH 1 Study to Evaluate Safety and Tolerability of XmAb14045 in Patients With CD123-expressing Hematologic Malignancies Recruiting NCT02730312 Phase 1
15 HA-1 T TCR T Cell Immunotherapy for the Treating of Patients With Relapsed or Refractory Acute Leukemia After Donor Stem Cell Transplant Recruiting NCT03326921 Phase 1 Fludarabine Phosphate
16 Rivogenlecleucel Donor Lymphocyte Immunotherapy in Treating Patients With Recurrent Blood Cancers After Stem Cell Transplant Not yet recruiting NCT03807063 Phase 1 Rimiducid
17 Descriptive Study of the Efficacy of Treatments for Blastic Dendritic Cell Neoplasm (BPDCN) Completed NCT02859623
18 Salvia Hispanica Seed in Reducing Risk of Disease Recurrence in Patients With Non-Hodgkin Lymphoma Active, not recruiting NCT02652715 Not Applicable
19 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 Not Applicable 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:

42
Nk Cells, Myeloid, T Cells, Bone, Skin, Lung, Bone Marrow

Publications for Blastic Plasmacytoid Dendritic Cell

Articles related to Blastic Plasmacytoid Dendritic Cell:

(show top 50) (show all 275)
# Title Authors Year
1
Blastic plasmacytoid dendritic cell neoplasm: genomics mark epigenetic dysregulation as a primary therapeutic target. ( 30381297 )
2019
2
Blastic plasmacytoid dendritic cell neoplasm with immunoblastoid morphology and MYC rearrangement and overexpression. ( 30482401 )
2019
3
Blastic plasmacytoid dendritic cell neoplasm with response to pralatrexate. ( 30652209 )
2019
4
Blastic Plasmacytoid Dendritic Cell Neoplasm; A Report of Three Cases. ( 30666080 )
2019
5
Clinical efficacy of bortezomib and lenalidomide in blastic plasmacytoid dendritic cell neoplasm. ( 30694364 )
2019
6
Blastic Plasmacytoid Dendritic Cell Neoplasm. ( 30715612 )
2019
7
Blastic plasmacytoid dendritic cell neoplasm with unusual extracutaneous manifestation: Two case reports and literature review. ( 30732162 )
2019
8
Blastic Plasmacytoid Dendritic Cell Neoplasm: A Case Report. ( 30734052 )
2019
9
More on Blastic Plasmacytoid Dendritic-Cell Neoplasms. ( 30763185 )
2019
10
Chronic myelomonocytic leukaemia followed by blastic plasmacytoid dendritic cell neoplasm. ( 30784042 )
2019
11
More on Blastic Plasmacytoid Dendritic-Cell Neoplasms. ( 30785714 )
2019
12
More on Blastic Plasmacytoid Dendritic-Cell Neoplasms. ( 30785715 )
2019
13
Blastic Plasmacytoid Dendritic Cell Neoplasm with leukemic component ( 30819627 )
2019
14
Blastic plasmacytoid dendritic cell neoplasm: an early presentation. ( 30865410 )
2019
15
Atraumatic splenic ruptures triggered both remission and death in a single case of blastic plasmacytoid dendritic cell neoplasm. ( 30918143 )
2019
16
Expounding on the essence of epigenetic and genetic abnormalities in blastic plasmacytoid dendritic cell neoplasms. ( 30930334 )
2019
17
A Unique Case of a Pediatric Patient With Blastic Plasmacytoid Dendritic Cell Neoplasm, Guillain Barre Syndrome, and Hemophagocytic Lymphohistiocytosis. ( 30933025 )
2019
18
Blastic plasmacytoid dendritic cell neoplasm with unusual lymphoid features and macrovacuoles. ( 30955062 )
2019
19
Lineage-specific RUNX2 super-enhancer activates MYC and promotes the development of blastic plasmacytoid dendritic cell neoplasm. ( 30971697 )
2019
20
Daratumumab for treatment of blastic plasmacytoid dendritic cell neoplasm A single-case report. ( 30975908 )
2019
21
Germline missense NF1 mutation in an elderly patient with a blastic plasmacytoid dendritic cell neoplasm. ( 30977107 )
2019
22
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) in leukaemic phase without skin lesions: a diagnostic and management challenge. ( 31003841 )
2019
23
Tagraxofusp in Blastic Plasmacytoid Dendritic-Cell Neoplasm. ( 31018069 )
2019
24
Blastic Plasmacytoid Dendritic Cell Neoplasm: State of the Art and Prospects. ( 31035408 )
2019
25
Successful outcome of second allogeneic bone marrow transplantation for blastic plasmacytoid dendritic cell neoplasm with MYC locus rearrangement. ( 31061790 )
2019
26
PD1/PD-L1 Expression in Blastic Plasmacytoid Dendritic Cell Neoplasm. ( 31109153 )
2019
27
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. ( 29876950 )
2019
28
A Case of Blastic Plasmacytoid Dendritic Cell Neoplasm with Unusual Presentation ( 30040070 )
2019
29
Clonal evolution in the transition from cutaneous disease to acute leukemia suggested by liquid biopsy in blastic plasmacytoid dendritic cell neoplasm. ( 29419438 )
2018
30
Incidental diagnosis of blastic plasmacytoid dendritic cell neoplasm in skin excision for basal cell carcinoma. ( 30094841 )
2018
31
Epidemiology and survival of blastic plasmacytoid dendritic cell neoplasm. ( 30189324 )
2018
32
Biallelic inactivation of the retinoblastoma gene results in transformation of chronic myelomonocytic leukemia to a blastic plasmacytoid dendritic cell neoplasm: shared clonal origins of two aggressive neoplasms. ( 30190511 )
2018
33
Genomic aberrations involving 12p/ETV6 are highly prevalent in blastic plasmacytoid dendritic cell neoplasms and might represent early clonal events. ( 30248580 )
2018
34
An unexpected diagnosis: leukaemic presentation of blastic plasmacytoid dendritic cell neoplasm with massive splenomegaly. ( 30274817 )
2018
35
Venetoclax in a Patient with a Blastic Plasmacytoid Dendritic-Cell Neoplasm. ( 30304659 )
2018
36
Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) on Social Media: #BPDCN-Increasing Exposure over Two Years Since Inception of a Disease-Specific Twitter Community. ( 30338458 )
2018
37
Blastic Plasmacytoid Dendritic Cell Neoplasm. ( 30350260 )
2018
38
Childhood Blastic Plasmacytoid Dendritic Cell Neoplasm Mimicking Acute Rheumatic Fever: Report of an Unusual Clinical Presentation and Review of Literature. ( 28859047 )
2018
39
Splenic Rupture Secondary to Blastic Plasmacytoid Dendritic Cell Neoplasm. ( 28963621 )
2018
40
A blastic plasmacytoid dendritic cell neoplasm-like phenotype identifies a subgroup of npm1-mutated acute myeloid leukemia patients with worse prognosis. ( 29080220 )
2018
41
Complete monocular vision loss due to optic nerve involvement of blastic plasmacytoid dendritic cell neoplasm. ( 29276838 )
2018
42
Blastic plasmacytoid dendritic cell neoplasm with centrocyte-like morphology clinically simulating a melanocytic nevus. ( 29293270 )
2018
43
Exploring the effect of chidamide on blastic plasmacytoid dendritic cell neoplasm: a case report and literature review. ( 29379294 )
2018
44
8q24/MYC rearrangement is a recurrent cytogenetic abnormality in blastic plasmacytoid dendritic cell neoplasms. ( 29407586 )
2018
45
Blastic plasmacytoid dendritic cell neoplasm with unusual morphology, MYC rearrangement and TET2 and DNMT3A mutations. ( 29411884 )
2018
46
Detection of Minimal Bone Marrow involvement of Blastic Plasmacytoid Dendritic Cell Neoplastic Cells - CD303 immunostaining as a diagnostic tool. ( 29415975 )
2018
47
Skin lesions serve as clues to relapse of pediatric blastic plasmacytoid dendritic cell neoplasm. ( 29436012 )
2018
48
Blastic plasmacytoid dendritic cell neoplasm: update on therapy especially novel agents. ( 29455234 )
2018
49
Immunophenotypic CD56 Variation Within a Single Patient With Blastic Plasmacytoid Dendritic Cell Neoplasm. ( 29490374 )
2018
50
Blastic plasmacytoid dendritic cell neoplasm. ( 29636962 )
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

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

# Super pathways Score Top Affiliating Genes
1 11.28 MYC NCAM1
2 10.82 CLEC4C NCAM1
3 10.41 DNMT3A MYC

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 cellular response to hypoxia GO:0071456 9.16 DNMT3A MYC
2 response to ethanol GO:0045471 8.96 DNMT3A MYC
3 response to estradiol GO:0032355 8.62 DNMT3A MYC

Sources for Blastic Plasmacytoid Dendritic Cell

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35 ICD10 via Orphanet
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63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
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75 UMLS via Orphanet
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