Blastic plasmacytoid dendritic cell neoplasm: a short review and update
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic neoplasm (less than 1% of primary cutaneous lymphomas and acute leukemia) with a highly aggressive clinical course and frequent skin, bone marrow and central nervous system (CNS) involvement. Despite a frequent initial respo...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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PAGEPress Publications
2023-08-01
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| Series: | Dermatology Reports |
| Subjects: | |
| Online Access: | https://pagepress.org/journals/index.php/dr/article/view/9781 |
| _version_ | 1797694026469605376 |
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| author | Cesare Massone Giulia Rivoli Simona Sola Emanuele Angelucci |
| author_facet | Cesare Massone Giulia Rivoli Simona Sola Emanuele Angelucci |
| author_sort | Cesare Massone |
| collection | DOAJ |
| description |
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic neoplasm (less than 1% of primary cutaneous lymphomas and acute leukemia) with a highly aggressive clinical course and frequent skin, bone marrow and central nervous system (CNS) involvement. Despite a frequent initial response to chemotherapy, relapses with eventual leukemic dissemination are extremely common, leading to poor outcomes and a median overall survival (OS) ranging from 8 to 14 months in first line setting, with standard combination chemotherapy regimens. The skin is the first affected site (in almost 90% of patients) where BPDCN may remain confined for weeks or even months (sanctuary?) until a rapid second step with multiorgan involvement occurs. Therefore, it is of uppermost importance to suspect and recognize early skin lesions and to perform and report a skin biopsy as soon as possible. A multidisciplinary approach with coordination among dermatologists, pathologists and hematologists is definitively crucial in diagnosis and management of BPDCN.
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| first_indexed | 2024-03-12T02:52:02Z |
| format | Article |
| id | doaj.art-3d7b0c9c8dc44e5ab528a7340658d10a |
| institution | Directory Open Access Journal |
| issn | 2036-7392 2036-7406 |
| language | English |
| last_indexed | 2024-03-12T02:52:02Z |
| publishDate | 2023-08-01 |
| publisher | PAGEPress Publications |
| record_format | Article |
| series | Dermatology Reports |
| spelling | doaj.art-3d7b0c9c8dc44e5ab528a7340658d10a2023-09-03T18:24:03ZengPAGEPress PublicationsDermatology Reports2036-73922036-74062023-08-0110.4081/dr.2021.9781Blastic plasmacytoid dendritic cell neoplasm: a short review and updateCesare Massone0Giulia Rivoli1Simona Sola2Emanuele Angelucci3Dermatology Unit, Galliera Hospital, GenoaUO Hematology and Cellular Therapies, IRCCS San Martino Polyclinic Hospital, GenoaSurgical Pathology, Galliera Hospital, GenoaUO Hematology and Cellular Therapies, IRCCS San Martino Polyclinic Hospital, Genoa Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic neoplasm (less than 1% of primary cutaneous lymphomas and acute leukemia) with a highly aggressive clinical course and frequent skin, bone marrow and central nervous system (CNS) involvement. Despite a frequent initial response to chemotherapy, relapses with eventual leukemic dissemination are extremely common, leading to poor outcomes and a median overall survival (OS) ranging from 8 to 14 months in first line setting, with standard combination chemotherapy regimens. The skin is the first affected site (in almost 90% of patients) where BPDCN may remain confined for weeks or even months (sanctuary?) until a rapid second step with multiorgan involvement occurs. Therefore, it is of uppermost importance to suspect and recognize early skin lesions and to perform and report a skin biopsy as soon as possible. A multidisciplinary approach with coordination among dermatologists, pathologists and hematologists is definitively crucial in diagnosis and management of BPDCN. https://pagepress.org/journals/index.php/dr/article/view/9781Blastic plasmacytoid dendritic cell neoplasmcutaneous lymphomatagraxofuspplasmacytoid dendritic cellsCD123 |
| spellingShingle | Cesare Massone Giulia Rivoli Simona Sola Emanuele Angelucci Blastic plasmacytoid dendritic cell neoplasm: a short review and update Dermatology Reports Blastic plasmacytoid dendritic cell neoplasm cutaneous lymphoma tagraxofusp plasmacytoid dendritic cells CD123 |
| title | Blastic plasmacytoid dendritic cell neoplasm: a short review and update |
| title_full | Blastic plasmacytoid dendritic cell neoplasm: a short review and update |
| title_fullStr | Blastic plasmacytoid dendritic cell neoplasm: a short review and update |
| title_full_unstemmed | Blastic plasmacytoid dendritic cell neoplasm: a short review and update |
| title_short | Blastic plasmacytoid dendritic cell neoplasm: a short review and update |
| title_sort | blastic plasmacytoid dendritic cell neoplasm a short review and update |
| topic | Blastic plasmacytoid dendritic cell neoplasm cutaneous lymphoma tagraxofusp plasmacytoid dendritic cells CD123 |
| url | https://pagepress.org/journals/index.php/dr/article/view/9781 |
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