CD4+/CD56+ hematodermic neoplasm
diseaseOn this page
Also known as agranular CD4+ CD56+ hematodermic neoplasm/tumoragranular CD4+ natural Killer cell leukaemiaagranular CD4+ natural Killer cell leukemiablastic natural Killer leukemia/lymphomablastic NK-cell lymphomablastic plasmacytoid dendritic cellblastic plasmacytoid Dendritic cell neoplasmBPDCNearly plasmacytoid Dendritic cell leukemia/lymphomalymphoblastoid variant of NK-cell lymphomamonomorphic NK-cell lymphomaprimary cutaneous CD4+/CD56+ hematolymphoid neoplasm
Summary
CD4+/CD56+ hematodermic neoplasm (MONDO:0019467) is a cancer and 38 clinical trials. Top therapeutic interventions include fludarabine phosphate, tagraxofusp, and ranitidine. A subtype of skin cancer — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Classification: Cancer
- Prevalence: 1-5 / 10 000 (Europe) [Orphanet-validated]
- Clinical trials: 38
Clinical features
Epidemiology
Prevalence records
2 prevalence record(s), Orphanet:
| Type | Class | Value | Geography | Validation |
|---|---|---|---|---|
| Point prevalence | 1-5 / 10 000 | 12 | Europe | Validated |
| Annual incidence | <1 / 1 000 000 | 0.03 | United States | Validated |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | CD4+/CD56+ hematodermic neoplasm |
| Mondo ID | MONDO:0019467 |
| EFO | EFO:0010580 |
| Orphanet | 86870 |
| DOID | DOID:0081076 |
| ICD-10-CM | C86.4 |
| ICD-11 | 783045723 |
| NCIT | C7203 |
| SNOMED CT | 445105005 |
| UMLS | C1301363 |
| MedGen | 220972 |
| GARD | 0010556 |
| Is cancer (heuristic) | yes |
Also known as: agranular CD4+ CD56+ hematodermic neoplasm/tumor · agranular CD4+ natural Killer cell leukaemia · agranular CD4+ natural Killer cell leukemia · blastic natural Killer leukemia/lymphoma · blastic NK-cell lymphoma · blastic plasmacytoid dendritic cell · blastic plasmacytoid Dendritic cell neoplasm · blastic plasmacytoid dendritic cell neoplasm · BPDCN · CD4+/CD56+ hematodermic neoplasm · early plasmacytoid Dendritic cell leukemia/lymphoma · lymphoblastoid variant of NK-cell lymphoma · monomorphic NK-cell lymphoma · primary cutaneous CD4+/CD56+ hematolymphoid neoplasm
Data availability: 5 cell lines.
Disease family
This is a subtype of skin cancer. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: disease › human disease › disease by body system or component › integumentary system disorder › integumentary system cancer › skin cancer › CD4+/CD56+ hematodermic neoplasm
Related subtypes (12): labium majus cancer, labia minora cancer, prepuce cancer, sweat gland cancer, cutaneous ganglioneuroma, skin carcinoma, malignant dermis tumor, skin meningioma, cutaneous melanoma, primary cutaneous lymphoma, eyelid cancer, sebaceous gland cancer
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
Drugs indicated for this disease
No approved or late-stage (phase ≥3) drug is indicated for this disease; the following are in earlier-phase trials only.
Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Cytarabine, Dexamethasone, Doxorubicin, Methotrexate, Methylprednisolone, Prednisone, Rituximab, Tagraxofusp, Venetoclax, Vincristine.
Clinical trials & evidence
Clinical trials
Clinical trials: 38.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE1 | 15 |
| PHASE2 | 11 |
| Not specified | 6 |
| PHASE1/PHASE2 | 5 |
| EARLY_PHASE1 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT03386513 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | Study of IMGN632 in Patients With Untreated BPDCN and Relapsed/Refractory BPDCN |
| NCT03779854 | PHASE2 | RECRUITING | Naive T Cell Depletion for Preventing Chronic Graft-versus-Host Disease in Children and Young Adults With Blood Cancers Undergoing Donor Stem Cell Transplant |
| NCT04216524 | PHASE2 | RECRUITING | Venetoclax, SL-401, and Chemotherapy for the Treatment of Blastic Plasmacytoid Dendritic Cell Neoplasm |
| NCT06006403 | PHASE1/PHASE2 | RECRUITING | Safety and Efficacy of CD123-targeted CAR-NK for Relapsed/Refractory Acute Myeloid Leukemia or Blastic Plasmacytoid Dendritic Cell Neoplasm |
| NCT06013423 | PHASE2 | RECRUITING | Cord Blood Transplant, Cyclophosphamide, Fludarabine, and Total-Body Irradiation in Treating Patients With High-Risk Hematologic Diseases |
| NCT07007052 | PHASE2 | NOT_YET_RECRUITING | Phase II Study Evaluating the Efficacy and Safety of the Combination of Tagraxofusp and Venetoclax in Treatment-naive Blastic Plasmacytoid Dendritic Cell Neoplasm Patients |
| NCT00397579 | PHASE1/PHASE2 | COMPLETED | DT388IL3 Fusion Protein in Treating Patients With Acute Myeloid Leukemia or Myelodysplastic Syndromes |
| NCT02113982 | PHASE1/PHASE2 | COMPLETED | Tagraxofusp in Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm or Acute Myeloid Leukemia |
| NCT02220985 | PHASE2 | COMPLETED | Selective Depletion of CD45RA+ T Cells From Allogeneic Peripheral Blood Stem Cell Grafts From HLA-Matched Related and Unrelated Donors in Preventing GVHD |
| NCT03075553 | PHASE2 | TERMINATED | Nivolumab in Treating Patients With Relapsed or Refractory Peripheral T-cell Lymphoma |
| NCT03246906 | PHASE2 | TERMINATED | Comparison of Triple GVHD Prophylaxis Regimens for Nonmyeloablative or Reduced Intensity Conditioning Unrelated Mobilized Blood Cell Transplantation |
| NCT03404193 | PHASE2 | TERMINATED | Venetoclax and Decitabine in Treating Participants With Relapsed/Refractory Acute Myeloid Leukemia or Relapsed High-Risk Myelodysplastic Syndrome |
| NCT03599960 | PHASE2 | UNKNOWN | Combination Chemotherapy in Patients With Newly Diagnosed BPDCN |
| NCT03739606 | PHASE2 | WITHDRAWN | Flotetuzumab in Treating Patients With Recurrent or Refractory CD123 Positive Blood Cancer |
| NCT04109482 | PHASE1/PHASE2 | TERMINATED | Trial to Evaluate the Safety and Efficacy of MB-102 in Patients With BPDCN. |
| NCT04317781 | PHASE2 | TERMINATED | Tagraxofusp in Treating Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm After Stem Cell Transplant |
| NCT02159495 | PHASE1 | ACTIVE_NOT_RECRUITING | Genetically Modified T-cell Immunotherapy in Treating Patients With Relapsed/Refractory Acute Myeloid Leukemia and Persistent/Recurrent Blastic Plasmacytoid Dendritic Cell Neoplasm |
| NCT03113643 | PHASE1 | RECRUITING | 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) |
| NCT03326921 | PHASE1 | RECRUITING | HA-1 T TCR T Cell Immunotherapy for the Treatment of Patients With Relapsed or Refractory Acute Leukemia After Donor Stem Cell Transplant |
| NCT04013685 | PHASE1 | ACTIVE_NOT_RECRUITING | Precision-T: A Study of Orca-T in Recipients Undergoing Allogeneic Transplantation for Hematologic Malignancies |
| NCT04318678 | PHASE1 | RECRUITING | CD123-Directed T-Cell Therapy for Acute Myelogenous Leukemia (CATCHAML) |
| NCT05362773 | PHASE1 | RECRUITING | A Study of MGD024 in Patients With Relapsed or Refractory Hematologic Malignancies |
| NCT05476770 | PHASE1 | RECRUITING | Tagraxofusp in Pediatric Patients With Relapsed or Refractory CD123 Expressing Hematologic Malignancies |
| NCT06690827 | PHASE1 | RECRUITING | Clinical Trial of CD123-targeted CAR-NK Therapy for Relapse/refractory AML or BPDCN |
| NCT06712810 | PHASE1 | RECRUITING | Q702 for the Treatment of Patients With Hematologic Malignancies |
| NCT01231919 | PHASE1 | COMPLETED | MK2206 in Treating Younger Patients With Recurrent or Refractory Solid Tumors or Leukemia |
| NCT02730312 | PHASE1 | COMPLETED | PH 1 Study to Evaluate Safety and Tolerability of XmAb14045 in Patients With CD123-expressing Hematologic Malignancies |
| NCT03203369 | PHASE1 | TERMINATED | Study to Evaluate the Safety and Clinical Activity of UCART123 in Patients With BPDCN |
| NCT03485547 | PHASE1 | COMPLETED | Study of Venetoclax, a BCL2 Antagonist, for Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) |
| NCT03807063 | PHASE1 | WITHDRAWN | Rivogenlecleucel Donor Lymphocyte Immunotherapy in Treating Patients With Recurrent Blood Cancers After Stem Cell Transplant |
| NCT04681105 | PHASE1 | COMPLETED | Flotetuzumab for the Treatment of Relapsed or Refractory Advanced CD123-Positive Hematological Malignancies |
| NCT06765876 | EARLY_PHASE1 | RECRUITING | CART123 T Cells in Relapsed or Refractory CD123+ Hematologic Malignancies: A Dose Escalation Phase I Trial |
| NCT05430971 | Not specified | RECRUITING | Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) International Registry |
| NCT00014235 | Not specified | COMPLETED | Fludarabine Phosphate and Total-Body Radiation Followed by Donor Peripheral Blood Stem Cell Transplant and Immunosuppression in Treating Patients With Hematologic Malignancies |
| NCT02652715 | Not specified | COMPLETED | Salvia Hispanica Seed in Reducing Risk of Disease Recurrence in Patients With Non-Hodgkin Lymphoma |
| NCT02859623 | Not specified | COMPLETED | Descriptive Study of the Efficacy of Treatments for Blastic Dendritic Cell Neoplasm (BPDCN) |
| NCT03974971 | Not specified | COMPLETED | Blastic Plasmacytoid Dendritic Cell Neoplasm in Korean Population. |
| NCT05645744 | Not specified | TERMINATED | Long-term Follow-up Study in Patients Previously Treated With a Mustang Bio CAR-T Cell Investigational Product. |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| FLUDARABINE PHOSPHATE | 4 | 8 |
| TAGRAXOFUSP | 4 | 7 |
| RANITIDINE | 4 | 1 |
| RELMAPIRAZIN | 3 | 1 |
| FLOTETUZUMAB | 2 | 1 |
| RIMIDUCID | 2 | 1 |
| RIVOGENLECLEUCEL | 2 | 1 |
| VIBECOTAMAB | 2 | 1 |
| PIVEKIMAB SUNIRINE | 1 | 1 |