Organ involvement in adults with BPDCN is associated with sun exposure history, TET2 and RAS mutations, and survival.

Autor:	Shimony S; Division of Hematologic Neoplasia, Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA.; Rabin Medical Center and Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel., Keating J; Department of Data Science, Dana-Farber Cancer Institute, Boston, MA., Fay CJ; Department of Dermatology, Brigham and Women's Hospital, Boston, MA.; Center for Cutaneous Oncology, Dana-Farber Brigham Cancer Center, Boston, MA., Luskin MR; Division of Hematologic Neoplasia, Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA., Neuberg DS; Department of Data Science, Dana-Farber Cancer Institute, Boston, MA., LeBoeuf NR; Department of Dermatology, Brigham and Women's Hospital, Boston, MA.; Center for Cutaneous Oncology, Dana-Farber Brigham Cancer Center, Boston, MA., Lane AA; Division of Hematologic Neoplasia, Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA.
Jazyk:	angličtina
Zdroj:	Blood advances [Blood Adv] 2024 Jun 11; Vol. 8 (11), pp. 2803-2812.
DOI:	10.1182/bloodadvances.2024012797
Abstrakt:	Abstract: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) can involve skin, bone marrow (BM), central nervous system (CNS), and non-CNS extramedullary sites. Preclinical models demonstrated clonal advantage of TET2-mutated plasmacytoid dendritic cells exposed to UV radiation. However, whether sun exposure, disease characteristics, and patient survival are clinically related is unclear. We classified organ involvement in 66 patients at diagnosis as skin only (n = 19), systemic plus skin (n = 33), or systemic only (n = 14). BM involvement was absent, microscopic (<5%), or overt (≥5%). UV exposure was based on clinical and demographic data. Patients with skin only BPDCN were more frequently aged ≥75 years (47% vs 19%; P = .032) and had lower rates of complex karyotype (0 vs 32%, P = .022) and mutated NRAS (0 vs 29%, P = .044). Conversely, those without skin involvement had lower UV exposure (23% vs 59%, P = .03) and fewer TET2 mutations (33% vs 72%, P = .051). The median overall survival (OS) was 23.5, 20.4, and 17.5 months for skin only, systemic plus skin, and systemic only, respectively. Patients with no BM involvement had better OS vs overt involvement (median OS, 27.3 vs 15.0 months; P = .033) and comparable with microscopic involvement (27.3 vs 23.5 months; P = .6). Overt BM involvement remained significant for OS when adjusted for baseline characteristics and treatment received. In summary, BPDCN clinical characteristics are associated with disease genetics and survival, which together may impact prognosis and indicate informative disease subtypes for future research. (© 2024 by The American Society of Hematology. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved.)
Databáze:	MEDLINE
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