Features of non-activation dendritic state and immune deficiency in blastic plasmacytoid dendritic cell neoplasm (BPDCN)
Autor: | Feng Wang, Naveen Pemmaraju, Li Zhao, Maliha Khan, Tianyu Cai, Hannah C. Beird, Marina Konopleva, Mansour Alfayez, P. Andrew Futreal, Joseph D. Khoury |
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Jazyk: | angličtina |
Rok vydání: | 2019 |
Předmět: |
Male
Microarray Protein Array Analysis Chronic myelomonocytic leukemia Context (language use) lcsh:RC254-282 Article Transcriptome 03 medical and health sciences 0302 clinical medicine Immune system hemic and lymphatic diseases Genetics research medicine Humans Leukaemia Genetic Predisposition to Disease Alleles Aged 030304 developmental biology Aged 80 and over 0303 health sciences business.industry Gene Expression Profiling Computational Biology Myeloid leukemia Dendritic Cells Hematology Middle Aged Eosinophil medicine.disease lcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogens 3. Good health Phenotype medicine.anatomical_structure Oncology Hematologic Neoplasms 030220 oncology & carcinogenesis Mutation Cancer research Female Disease Susceptibility Bone marrow business |
Zdroj: | Blood Cancer Journal, Vol 9, Iss 12, Pp 1-9 (2019) Blood Cancer Journal |
ISSN: | 2044-5385 |
Popis: | Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, male-predominant hematologic malignancy with poor outcomes and with just one recently approved agent (tagraxofusp). It is characterized by the abnormal proliferation of precursor plasmacytoid dendritic cells (pDCs) with morphologic and molecular similarities to acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS)/chronic myelomonocytic leukemia (CMML) in its presentation within the bone marrow and peripheral blood. To identify disease-specific molecular features of BPDCN, we profiled the bone marrow, peripheral blood, and serum samples from primary patient samples using an in-house hematologic malignancy panel (“T300” panel), transcriptome microarray, and serum multiplex immunoassays. TET2 mutations (5/8, 63%) were the most prevalent in our cohort. Using the transcriptome microarray, genes specific to pDCs (LAMP5, CCDC50) were more highly expressed in BPDCN than in AML specimens. Finally, the serum cytokine profile analysis showed significantly elevated levels of eosinophil chemoattractants eotaxin and RANTES in BPDCN as compared with AML. Along with the high levels of PTPRS and dendritic nature of the tumor cells, these findings suggest a possible pre-inflammatory context of this disease, in which BPDCN features nonactivated pDCs. |
Databáze: | OpenAIRE |
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