Disease · MONDO_0019467

CD4+/CD56+ hematodermic neoplasm

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. CD4+/CD56+ hematodermic neoplasm. Diseases, record MONDO_0019467. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0019467

On this page

Disease

Names and IDs as Open Targets gives them. Its IDs in MeSH, Orphanet, the Disease Ontology and the NCI Thesaurus are those Open Targets states for it.

Name: CD4+/CD56+ hematodermic neoplasm; Open Targets
Open Targets ID: MONDO_0019467; Open Targets
Other names: BPDCN, agranular CD4+ CD56+ hematodermic neoplasm/tumor, agranular CD4+ natural Killer cell leukaemia, agranular CD4+ natural Killer cell leukemia, blastic NK-cell lymphoma, blastic natural Killer leukemia/lymphoma, blastic plasmacytoid Dendritic cell neoplasm, early plasmacytoid Dendritic cell leukemia/lymphoma, lymphoblastoid variant of NK-cell lymphoma, monomorphic NK-cell lymphoma, primary cutaneous CD4+/CD56+ hematolymphoid neoplasm; Open Targets
Disease Ontology: DOID:0081076; Disease Ontology
NCI Thesaurus: C7203; NCI Thesaurus
Orphanet: ORPHA:86870; Orphanet
Highest stage of its drugs: Approval, for 1 of its 18 drugs, from TTD; Open Targets

Drugs18

The drugs Open Targets' clinical reports study for the disease, the highest stage first, then the most trial reports. The stage is Open Targets', the highest any of its sources reports for the pair, and each row names the sources that report it.

Drug	ChEMBL ID	Type	Open Targets' stage	Stage reported by	Reports	Of them trials	Trials shown here	Source
TAGRAXOFUSP	CHEMBL4297573	Protein	Approval	TTD	7	5	5	Drugs and targets
VENETOCLAX	CHEMBL3137309	Small molecule	Phase 2	ClinicalTrials.gov	5	5	5	Drugs and targets
CYCLOPHOSPHAMIDE	CHEMBL1200796	Small molecule	Phase 2	ClinicalTrials.gov	3	3	3	Drugs and targets
DEXAMETHASONE	CHEMBL384467	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
METHOTREXATE	CHEMBL34259	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
ASPARAGINASE	CHEMBL2108989	Enzyme	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
CYTARABINE	CHEMBL803	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
DECITABINE	CHEMBL1201129	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
DOXORUBICIN	CHEMBL53463	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
IDARUBICIN	CHEMBL1117	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets

1 to 10 of 18

See all 18 drugs

Targets37

The targets ChEMBL's mechanism records give for the disease's drugs, through Open Targets, the most drugs first. A mechanism record says what a drug acts on, not that the target causes the disease.

Target	Protein	Its drugs that act on it	Mechanisms	Highest stage of those drugs	Gene in Genes	Source
DNMT1	DNA (cytosine-5)-methyltransferase 1	2	DNA (cytosine-5)-methyltransferase 1 inhibitor	Phase 2	https://conductscience.org/library/genes/1786	Drugs and targets
DNMT3A	DNA (cytosine-5)-methyltransferase 3A	2	DNA (cytosine-5)-methyltransferase 3A inhibitor	Phase 2	https://conductscience.org/library/genes/1788	Drugs and targets
IL3RA	Interleukin-3 receptor subunit alpha	2	Interleukin-3 receptor subunit alpha binding agent	Approval	https://conductscience.org/library/genes/3563	Drugs and targets
IL3RA	Interleukin-3 receptor subunit alpha	2	Interleukin-3 receptor subunit alpha binding agent	Approval	https://conductscience.org/library/genes/3563	Drugs and targets
NR3C1	Glucocorticoid receptor	2	Glucocorticoid receptor agonist	Phase 2	https://conductscience.org/library/genes/2908	Drugs and targets
TOP2A	DNA topoisomerase 2-alpha	2	DNA topoisomerase II alpha inhibitor	Phase 2	https://conductscience.org/library/genes/7153	Drugs and targets
BCL2	Apoptosis regulator Bcl-2	1	Apoptosis regulator Bcl-2 inhibitor	Phase 2	https://conductscience.org/library/genes/596	Drugs and targets
DHFR	Dihydrofolate reductase	1	Dihydrofolate reductase inhibitor	Phase 2	https://conductscience.org/library/genes/1719	Drugs and targets
EEF2	Elongation factor 2	1	Elongation factor 2 inhibitor	Approval	https://conductscience.org/library/genes/1938	Drugs and targets
MS4A1	B-lymphocyte antigen CD20	1	B-lymphocyte antigen CD20 binding agent	Phase 2	https://conductscience.org/library/genes/931	Drugs and targets

1 to 10 of 37

See all 37 targets

Trials11

Trials an Open Targets clinical report links to the disease, posted results first, then the largest. A trial is shown when the release's checks confirm its link to the disease and Drugs and targets shows its link to one of the disease's drugs.

Trial	Title	Phase	Participants	Results first posted	Conditions	Drugs	Linked by	Source
NCT02113982	Tagraxofusp in Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm or Acute Myeloid Leukemia	Phase 1/2	138	2024-08-01	Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN), Acute Myeloid Leukemia (AML)	TAGRAXOFUSP	Its record names the disease	ClinicalTrials.gov
NCT02623439	A Study of Haploidentical Bone Marrow Transplant for Patients With Hematologic Malignancies	Phase 2	10	2025-11-10	Hematologic Malignancies	CYCLOPHOSPHAMIDE	Its record names the disease	ClinicalTrials.gov
NCT04317781	Tagraxofusp in Treating Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm After Stem Cell Transplant	Phase 2	3	2024-07-16	Blastic Plasmacytoid Dendritic Cell Neoplasm	TAGRAXOFUSP	Its record names the disease	ClinicalTrials.gov
NCT03404193	Venetoclax and Decitabine in Treating Participants With Relapsed/Refractory Acute Myeloid Leukemia or Relapsed High-Risk Myelodysplastic Syndrome	Phase 2	235		Acute Myeloid Leukemia, Acute Myeloid Leukemia Arising From Previous Myelodysplastic Syndrome, Blastic Plasmacytoid Dendritic Cell Neoplasm, Chronic Myelomonocytic Leukemia, Mixed Phenotype Acute Leukemia, Myelodysplastic Syndrome, Recurrent Acute Biphenotypic Leukemia, Recurrent Acute Myeloid Leukemia, Recurrent Blastic Plasmacytoid Dendritic Cell Neoplasm, Recurrent Chronic Myelomonocytic Leukemia, Recurrent Mixed Phenotype Acute Leukemia, Refractory Acute Myeloid Leukemia, Refractory Blastic Plasmacytoid Dendritic Cell Neoplasm, Refractory Chronic Myelomonocytic Leukemia, Refractory Mixed Phenotype Acute Leukemia	DECITABINE, VENETOCLAX	Its record names the disease	ClinicalTrials.gov
NCT04318678	CD123-Directed T-Cell Therapy for Acute Myelogenous Leukemia (CATCHAML)	Phase 1	108		AML/MDS, B-ALL, T-ALL, BPDCN	CYCLOPHOSPHAMIDE, FLUDARABINE	Its record names the disease	ClinicalTrials.gov
NCT03113643	SL-401 in Combination With Azacitidine or Azacitidine/Venetoclax in Acute Myeloid Leukemia (AML), High-Risk Myelodysplastic Syndrome (MDS) or Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN)	Phase 1	72		Acute Myeloid Leukemia, Myelodysplastic Syndrome, Blastic Plasmacytoid Dendritic Cell Neoplasm	AZACITIDINE, TAGRAXOFUSP, VENETOCLAX	Its record names the disease	ClinicalTrials.gov
NCT04216524	Venetoclax, SL-401, and Chemotherapy for the Treatment of Blastic Plasmacytoid Dendritic Cell Neoplasm	Phase 2	40		Blastic Plasmacytoid Dendritic Cell Neoplasm	CYCLOPHOSPHAMIDE, CYTARABINE, DEXAMETHASONE, DOXORUBICIN, MERCAPTOPURINE, METHOTREXATE, METHYLPREDNISOLONE, RITUXIMAB, TAGRAXOFUSP, VENETOCLAX, VINCRISTINE	Its record names the disease	ClinicalTrials.gov
NCT07007052	Phase II Study Evaluating the Efficacy and Safety of the Combination of Tagraxofusp and Venetoclax in Treatment-naive Blastic Plasmacytoid Dendritic Cell Neoplasm Patients	Phase 2	33		Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN)	TAGRAXOFUSP, VENETOCLAX	Its record names the disease	ClinicalTrials.gov
NCT03599960	Combination Chemotherapy in Patients With Newly Diagnosed BPDCN	Phase 2	26		Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN)	ASPARAGINASE, DEXAMETHASONE, IDARUBICIN, METHOTREXATE	Its record names the disease	ClinicalTrials.gov
NCT03485547	Study of Venetoclax, a BCL2 Antagonist, for Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN)	Phase 1	5		Blastic Plasmacytoid Dendritic Cell Neoplasm	VENETOCLAX	Its record names the disease	ClinicalTrials.gov

1 to 10 of 11

See all 11 trials

Comparisons in its trials0

Comparisons Trial comparisons calculated in the trials shown here, most participants first. A comparison in one of these trials does not always compare a treatment for the disease, so read the two groups.

None in this release.

Papers from its trials12

The papers the trials shown here list on ClinicalTrials.gov as their results (RESULT) or as drawn from them (DERIVED), results first, newest first.

PMID	Listed as	Trials listing it	Source
42413008	DERIVED	1	PubMed
41486505	DERIVED	1	PubMed
38052038	DERIVED	1	PubMed
35820082	DERIVED	1	PubMed
34474640	DERIVED	1	PubMed
34255353	DERIVED	1	PubMed
33795208	DERIVED	3	PubMed
33792630	DERIVED	1	PubMed
33580980	DERIVED	1	PubMed
33027528	DERIVED	1	PubMed

1 to 10 of 12

See all 12 papers from its trials

Data dates

The date of each source this release read.

Open Targets: 2026-09-18; Open Targets
Drug links and their checks, from Drugs and targets: 2026-09-18; Drugs and targets
ClinicalTrials.gov, from Trials: 2026-11-12; Trials
Comparisons, from Trial comparisons: 2026-03-23; Trial comparisons
PubMed, for the checks: 2026-11-06; PubMed
MeSH, for the checks: 2026-11-10; MeSH

Sources 6

ClinicalTrials.gov's posted results, through CTTI's AACTFreely available. Acknowledgement requested
ChEMBL, through Open TargetsCC BY-SA 3.0
ClinicalTrials.govNo license stated
NLM's MeSHNLM's terms
Open TargetsCC0 1.0
PubMedNLM's terms

Each source's terms

ClinicalTrials.gov's posted results, through CTTI's AACTClinicalTrials.gov data via Aggregate Analysis of ClinicalTrials.gov (AACT), CTTI. CTTI makes its materials freely available and requests acknowledgement. https://aact.ctti-clinicaltrials.org/documentation/299
ChEMBL, through Open TargetsCC BY-SA 3.0, for the parts from ChEMBL. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
ClinicalTrials.govnone stated
NLM's MeSHNLM's
Open TargetsCC0 1.0. Open Targets asks that users cite its latest paper: Buniello et al. 2025, Open Targets Platform: facilitating therapeutic hypotheses building in drug discovery, Nucleic Acids Research 53(D1):D1467, PMID 39657122. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
PubMedNLM's terms

ClinicalTrials.gov's data carry an international copyright outside the United States: https://clinicaltrials.gov/about-site/terms-conditions.

For software

Read this disease from software.

The record and each of its lists are JSON, twenty rows a page. No key.

GET https://conductscience.org/api/diseases/v1/MONDO_0019467

GET https://conductscience.org/api/diseases/v1/MONDO_0019467/targets?page=2

API reference For AI assistants