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GENE:

CLEC4C (C-Type Lectin Domain Family 4 Member C)

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Other names: CLEC4C, C-Type Lectin Domain Family 4 Member C, BDCA2, HECL, DLEC, CLECSF11, CLECSF7, CD303, C-Type (Calcium Dependent, Carbohydrate-Recognition Domain) Lectin, Superfamily Member 7, C-Type Lectin Superfamily Member 7, Dendritic Lectin, BDCA-2, C-Type (Calcium Dependent, Carbohydrate-Recognition Domain) Lectin, Superfamily Member 11, C-Type Lectin Domain Family 4, Member C, Blood Dendritic Cell Antigen 2 Protein, Blood Dendritic Cell Antigen 2, Dendritic Cell Lectin B, CD303 Antigen, PRO34150
Associations
10d
Dual Immunological Prognostic Models for Risk Stratification and Treatment Insights in Triple-Negative Breast Cancer. (PubMed, Int J Mol Sci)
Furthermore, we identified several potential therapeutic agents, including imatinib and bortezomib. Collectively, our dual-model framework provides a tool for risk stratification, offers translational insights for precision treatment, and presents new directions for understanding TNBC heterogeneity and therapeutic development.
Journal • IO biomarker
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NECTIN4 (Nectin Cell Adhesion Molecule 4) • CLEC4C (C-Type Lectin Domain Family 4 Member C) • NECTIN1 (Nectin Cell Adhesion Molecule 1)
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imatinib • bortezomib
2ms
The role of targeted therapies in blastic plasmacytoid dendritic cell neoplasm. (PubMed, Expert Opin Biol Ther)
The approval of tagraxofusp established a foundation for targeted therapy in BPDCN and demonstrated high response rates, leading to the development of additional antibody - drug conjugates and cellular therapies targeting CD123. Other promising targeted approaches include BCL2 inhibitors, proteasome inhibitors, and therapies directed against surface antigens such as CD38 and CD303, as well as bromodomain and extraterminal (BET) inhibitors. Collectively, these strategies represent important advances that may significantly improve the historically poor outcomes associated with BPDCN.
Review • Journal
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CD123 (Interleukin 3 Receptor Subunit Alpha) • IL3RA (Interleukin 3 Receptor Subunit Alpha) • CLEC4C (C-Type Lectin Domain Family 4 Member C)
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Venclexta (venetoclax) • Elzonris (tagraxofusp-erzs)
2ms
[89Zr]Zr-DFO-avelumab accumulation in resectable non-small cell lung carcinoma indicates a suppressive tumor microenvironment amendable to neoadjuvant avelumab treatment. (PubMed, Immunother Adv)
[89Zr]Zr-DFO-avelumab PET imaging is a safe and feasible approach in early-stage NSCLC. Higher [89Zr]Zr-DFO-avelumab tumor-accumulation at baseline strongly correlates with features of a suppressive tumor immune environment and response to neoadjuvant avelumab.
Journal • PD(L)-1 Biomarker • IO biomarker
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PD-L1 (Programmed death ligand 1) • CLEC4C (C-Type Lectin Domain Family 4 Member C)
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PD-L1 expression
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Bavencio (avelumab)
4ms
Plasmacytoid Dendritic Cell-Expansion in Acute Myeloid Leukemia (pDC‑AML): a Review of Clinicopathologic Features, Genetics, and Outcomes. (PubMed, Curr Hematol Malig Rep)
Reports on venetoclax-based therapy show mixed outcomes, while CD123-directed therapies are being explored...Early recognition, dual-compartment minimal residual disease assessment, timely transplant consideration, and referral for clinical trials are central to management. Standardized immunophenotypic criteria, integrated genomic profiling, and prospective evaluation of CD123-targeted and rational combination therapies are priorities to improve classification, risk stratification, and patient outcomes.
Review • Journal
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FLT3 (Fms-related tyrosine kinase 3) • NRAS (Neuroblastoma RAS viral oncogene homolog) • KIT (KIT proto-oncogene, receptor tyrosine kinase) • DNMT3A (DNA methyltransferase 1) • RUNX1 (RUNX Family Transcription Factor 1) • ASXL1 (ASXL Transcriptional Regulator 1) • TET2 (Tet Methylcytosine Dioxygenase 2) • CD123 (Interleukin 3 Receptor Subunit Alpha) • CD34 (CD34 molecule) • IL3RA (Interleukin 3 Receptor Subunit Alpha) • NRP1 (Neuropilin 1) • CLEC4C (C-Type Lectin Domain Family 4 Member C)
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NRAS mutation • RUNX1 mutation • TET2 mutation
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Venclexta (venetoclax)
6ms
Real-world management of blastic plasmacytoid dendritic cell neoplasm at an academic center with a broad regional referral base. (PubMed, Leuk Res Rep)
In this case series, we describe our institutional experience treating BPDCN both pre- and post-tagraxofusp approval. We summarize six cases, the majority of which occurred in elderly patients, and highlight unique challenges of treating BPDCN at a center that serves a large rural/frontier area with variable access to specialty care.
Journal • Real-world evidence
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CD123 (Interleukin 3 Receptor Subunit Alpha) • CD4 (CD4 Molecule) • NCAM1 (Neural cell adhesion molecule 1) • IL3RA (Interleukin 3 Receptor Subunit Alpha) • NRP1 (Neuropilin 1) • CLEC4C (C-Type Lectin Domain Family 4 Member C) • TCF4 (Transcription Factor 4)
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Elzonris (tagraxofusp-erzs)
6ms
Hematopathological profile of plasmacytoid dendritic cell proliferation associated with non-myeloid acute leukemia. (PubMed, Cytometry B Clin Cytom)
PDCs and blast cells shared some lymphoid antigens that were uncommon in AML-pDC or BPDCN. In the bone marrow, pDCs were predominantly characterized by an interstitial infiltration pattern.
Journal
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NRAS (Neuroblastoma RAS viral oncogene homolog) • CD19 (CD19 Molecule) • CD34 (CD34 molecule) • NCAM1 (Neural cell adhesion molecule 1) • CD7 (CD7 Molecule) • NRP1 (Neuropilin 1) • CLEC4C (C-Type Lectin Domain Family 4 Member C)
7ms
Single cell sequencing deciphering the heterogeneous landscape of blastic plasmacytoid dendritic cell neoplasm with novel MYB-ZFAT fusion gene. (PubMed, Cancer Cell Int)
The study indicates BPDCN's complexity with varied cellular origins and a significant role for MYB activation in its development. This research deepens our comprehension of BPDCN's pathogenesis and cell populations.
Journal • IO biomarker
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CD123 (Interleukin 3 Receptor Subunit Alpha) • CD4 (CD4 Molecule) • NCAM1 (Neural cell adhesion molecule 1) • IL3RA (Interleukin 3 Receptor Subunit Alpha) • NRP1 (Neuropilin 1) • CLEC4C (C-Type Lectin Domain Family 4 Member C)
9ms
Nodal Mature Plasmacytoid Dendritic Cell Proliferation in a Patient With Chronic Myelomonocytic Leukemia: A Diagnostic Mimic of Blastic Plasmacytoid Dendritic Cell Neoplasm. (PubMed, J Investig Med High Impact Case Rep)
The patient was initially treated with hydroxyurea, later transitioned to decitabine/cedazuridine (Inqovi) for disease progression. Recognizing MPDCP in unusual locations is critical for accurate diagnosis and prognostication. Further studies are warranted to clarify its molecular pathogenesis and potential as a biomarker of disease evolution in CMML and related disorders.
Journal
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ASXL1 (ASXL Transcriptional Regulator 1) • CD123 (Interleukin 3 Receptor Subunit Alpha) • CD4 (CD4 Molecule) • NCAM1 (Neural cell adhesion molecule 1) • GZMB (Granzyme B) • IL3RA (Interleukin 3 Receptor Subunit Alpha) • CLEC4C (C-Type Lectin Domain Family 4 Member C)
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ASXL1 mutation • CBL mutation
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Inqovi (decitabine/cedazuridine) • hydroxyurea
11ms
Immunotherapies Targeting CD123 and CD303: A New Frontier in Treating Blastic Plasmacytoid Dendritic Cell Neoplasm. (PubMed, Int J Mol Sci)
Tagraxofusp, the first FDA-approved CD123-targeted therapy, has significantly improved patient outcomes...This review explores the latest developments in BPDCN treatment, emphasizing the potential of CD123 and CD303 as targets for precision medicine interventions. The ongoing evolution of targeted immunotherapies holds promise for improving patient survival and redefining treatment paradigms in hematologic malignancies.
Review • Journal • IO biomarker
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CD123 (Interleukin 3 Receptor Subunit Alpha) • IL3RA (Interleukin 3 Receptor Subunit Alpha) • CLEC4C (C-Type Lectin Domain Family 4 Member C)
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Elzonris (tagraxofusp-erzs)
1year
Identification of a Novel Immune-Gene Signature with Prognostic Value in Patients with Head and Neck Cancer: A Pilot Study. (PubMed, Biochem Genet)
By performing analyses of HNSCC tumor samples from The Cancer Genome Atlas (TCGA) database, we discovered that decreased expression of these genes, both as single genes and as a 5-gene signature (5-GS), was significantly correlated with worse overall survival (OS). Our data show that the levels of expression of the 5-GS represent an immune profile predicting OS in patients with HNSCC.
Journal • Gene Signature
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LY9 (Lymphocyte Antigen 9) • IL23A (Interleukin 23 Subunit Alpha) • LCK (LCK Proto-Oncogene, Src Family Tyrosine Kinase) • CLEC4C (C-Type Lectin Domain Family 4 Member C)
1year
The Challenge of Diagnosing Acute Myeloid Leukemia or Blastic Plasmacytoid Dendritic Cell Neoplasm: A Case Report. (PubMed, Anticancer Res)
AML with monocytic differentiation can express CD4, CD56, and CD123, which are very often the only markers considered for diagnosis of BPDCN. An extended panel of IHC analysis is required before making a definitive diagnosis of BPDCN.
Journal
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KIT (KIT proto-oncogene, receptor tyrosine kinase) • CD123 (Interleukin 3 Receptor Subunit Alpha) • CD34 (CD34 molecule) • NCAM1 (Neural cell adhesion molecule 1) • IL3RA (Interleukin 3 Receptor Subunit Alpha) • CLEC4C (C-Type Lectin Domain Family 4 Member C)
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CD123 positive • NCAM1 expression • CD4 expression • IL3RA positive
1year
Novel Prognostic Markers for Skin Cutaneous Melanoma. (PubMed, Clin Cosmet Investig Dermatol)
Subsequent knockdown of IL4I1 promoted both the proliferation and inhibited the apoptosis of melanoma cells. In summary, our study identified a series of potential prognostic genes in melanoma and verified the functional role of IL4I1 among them.
Journal
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CCR8 (C-C Motif Chemokine Receptor 8) • PLAC8 (Placenta Associated 8) • CLEC4C (C-Type Lectin Domain Family 4 Member C) • IL4I1 (Interleukin 4 Induced 1)