Details of Disease

General Information of Disease (ID: DISLEJU7)

• Disease Name: Blastic plasmacytoid dendritic cell neoplasm
• Synonyms: blastic plasmacytoid dendritic cell; early plasmacytoid Dendritic cell leukemia/lymphoma; blastic natural Killer leukemia/lymphoma; agranular CD4+ natural Killer cell leukaemia; CD4+/CD56+ hematodermic neoplasm; blastic plasmacytoid dendritic cell neoplasm; lymphoblastoid variant of NK-cell lymphoma; agranular CD4+ natural Killer cell leukemia; blastic NK-cell lymphoma; primary cutaneous CD4+/CD56+ hematolymphoid neoplasm; monomorphic NK-cell lymphoma; BPDCN; agranular CD4+ CD56+ hematodermic neoplasm/tumor; blastic plasmacytoid Dendritic cell neoplasm
• Disease Class: 2A60: Acute myeloid leukaemia
• Definition: An aggressive immature hematologic neoplasm formerly known as blastic NK cell lymphoma, composed of cells with a lymphoblast-like morphology. Recent evidence suggests derivation from a plasmacytoid monocyte. Patients present with cutaneous tumors and bone marrow involvement.
• Disease Hierarchy:
  DISSXRTQ: T-cell lymphoma
  DIS2YOWO: Myeloid neoplasm
  DISTM18U: Skin cancer
  DISLEJU7: Blastic plasmacytoid dendritic cell neoplasm
• ICD Code:
  ICD-11: ICD-11: 2A60.5
  ICD-10: ICD-10: C86.4
• Disease Identifiers:
  MONDO ID: MONDO_0019467
  UMLS CUI: C1301363
  MedGen ID: 220972
  Orphanet ID: 86870
  SNOMED CT ID: 445030005

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 1 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Tagraxofusp	DM9HQ5U	Approved	NA	[1]

This Disease is Treated as An Indication in 3 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
IMGN632	DM3KCIP	Phase 1/2	NA	[2]
SL-401	DMOCHVE	Phase 1/2	NA	[3]
UCART123	DMG7FLI	Phase 1	CAR T Cell Therapy	[4]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 4 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
CLEC4C	TT7YT06	moderate	Biomarker	[5]
NCAM1	TTVXPHT	moderate	Altered Expression	[6]
PTPRS	TTCWXFA	Strong	Altered Expression	[7]
IL3RA	TTENHJ0	Definitive	Biomarker	[8]

This Disease Is Related to 5 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
IL3	OT0CQ35N	Limited	Biomarker	[9]
CCDC50	OTSE1YM5	Strong	Altered Expression	[7]
CLTC	OTBFASMA	Strong	Biomarker	[10]
LAMP5	OTC672RM	Strong	Altered Expression	[7]
MLLT1	OT41UZU5	Strong	Genetic Variation	[11]

References

• [1] 2018 FDA drug approvals.Nat Rev Drug Discov. 2019 Feb;18(2):85-89.
• [2] ClinicalTrials.gov (NCT03386513) Study of IMGN632 in Patients With Untreated BPDCN and Relapsed/Refractory BPDCN. U.S. National Institutes of Health.
• [3] ClinicalTrials.gov (NCT02113982) SL-401 in Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm or Acute Myeloid Leukemia. U.S. National Institutes of Health.
• [4] Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
• [5] Clinicopathological analysis of 46 cases with CD4(+) and/or CD56(+) immature haematolymphoid malignancy: reappraisal of blastic plasmacytoid dendritic cell and related neoplasms.Histopathology. 2017 Dec;71(6):972-984. doi: 10.1111/his.13340. Epub 2017 Oct 10.
• [6] Blastic plasmacytoid dendritic cell neoplasm: update on therapy especially novel agents.Ann Hematol. 2018 Apr;97(4):563-572. doi: 10.1007/s00277-018-3259-z. Epub 2018 Feb 17.
• [7] Features of non-activation dendritic state and immune deficiency in blastic plasmacytoid dendritic cell neoplasm (BPDCN).Blood Cancer J. 2019 Dec 6;9(12):99. doi: 10.1038/s41408-019-0262-0.
• [8] Blastic Plasmacytoid Dendritic Cell Neoplasm.Curr Treat Options Oncol. 2019 Feb 4;20(1):9. doi: 10.1007/s11864-019-0605-x.
• [9] Tagraxofusp: First Global Approval.Drugs. 2019 Apr;79(5):579-583. doi: 10.1007/s40265-019-01087-z.
• [10] CLTC-ALK fusion as a primary event in congenital blastic plasmacytoid dendritic cell neoplasm.Genes Chromosomes Cancer. 2014 Jan;53(1):78-89. doi: 10.1002/gcc.22119. Epub 2013 Oct 21.
• [11] KMT2A (MLL)-MLLT1 rearrangement in blastic plasmacytoid dendritic cell neoplasm.Cancer Genet. 2015 Sep;208(9):464-7. doi: 10.1016/j.cancergen.2015.04.011. Epub 2015 May 6.