Academic Journal
Blastic plasmacytoid dendritic cell neoplasm: a short review and update
| العنوان: | Blastic plasmacytoid dendritic cell neoplasm: a short review and update |
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| المؤلفون: | Cesare Massone, Giulia Rivoli, Simona Sola, Emanuele Angelucci |
| المصدر: | Dermatology Reports (2023) |
| بيانات النشر: | PAGEPress Publications, 2023. |
| سنة النشر: | 2023 |
| المجموعة: | LCC:Dermatology |
| مصطلحات موضوعية: | Blastic plasmacytoid dendritic cell neoplasm, cutaneous lymphoma, tagraxofusp, plasmacytoid dendritic cells, CD123, Dermatology, RL1-803 |
| الوصف: | 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. |
| نوع الوثيقة: | article |
| وصف الملف: | electronic resource |
| اللغة: | English |
| تدمد: | 2036-7392 2036-7406 |
| Relation: | https://pagepress.org/journals/index.php/dr/article/view/9781; https://doaj.org/toc/2036-7392; https://doaj.org/toc/2036-7406 |
| DOI: | 10.4081/dr.2021.9781 |
| URL الوصول: | https://doaj.org/article/3d7b0c9c8dc44e5ab528a7340658d10a |
| رقم الانضمام: | edsdoj.3d7b0c9c8dc44e5ab528a7340658d10a |
| قاعدة البيانات: | Directory of Open Access Journals |
Full Text Finder | تدمد: | 20367392 20367406 |
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| DOI: | 10.4081/dr.2021.9781 |