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Unmet Clinical Needs and Management Recommendations for Blastic Plasmacytoid Dendritic Cell Neoplasm: A Consensus-based Position Paper From an Ad Hoc International Expert Panel

Authors :
Livio Pagano
Pier Luigi Zinzani
Stefano Pileri
Pietro Quaglino
Branko Cuglievan
Emilio Berti
Naveen Pemmaraju
Francesco Onida
Rein Willemze
Alberto Orfao
Giovanni Barosi
Source :
HemaSphere, Vol 7, Iss 3, p e841 (2023)
Publication Year :
2023
Publisher :
Wiley, 2023.

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a hematological malignancy characterized by recurrent skin nodules, an aggressive clinical course with rapid involvement of hematological organs, and a poor prognosis with overall survival. The rarity of the disease results in a few large-scale studies, a lack of controlled clinical trials for its management, and a lack of evidence-based guidelines. Here, we present a review of unmet clinical needs on the management of BPDCN by a panel of eleven experts involved in the research and clinical practice of BPDCN. Recommendations and proposals were achieved by multiple-step formalized procedures to reach a consensus after a comprehensive analysis of the scientific literature. The panel analyzed the critical issues of diagnostic pathway, prognostic stratification, therapy for young and fit patients and elderly and unfit patients, indication for allotransplant and for autotransplant, indication for central nervous system prophylaxis, and management of pediatric BPDCN patients. For each of these issues, consensus opinions were provided and, when appropriate, proposals for advancement in clinical practice were addressed. The hope is that this comprehensive overview will serve to improve the practice of BPDCN and inform the design and implementation of new studies in the field.

Details

Language :
English
ISSN :
25729241 and 00000000
Volume :
7
Issue :
3
Database :
Directory of Open Access Journals
Journal :
HemaSphere
Publication Type :
Academic Journal
Accession number :
edsdoj.fb8eb1b6e44bf9ae63db66febb6bd0
Document Type :
article
Full Text :
https://doi.org/10.1097/HS9.0000000000000841