Blastic Plasmacytoid Dendritic Cell Neoplasm, from a Dermatological Point of View

被引：1

作者：

Di Raimondo, Cosimo ^{[1
]}

Lozzi, Flavia ^{[2
]}

Di Domenico, Pier Paolo ^{[2
]}

Paganini, Claudia ^{[2
]}

Campione, Elena ^{[1
,2
]}

Galluzzo, Marco ^{[1
,2
]}

Bianchi, Luca ^{[1
,2
]}

机构：

[1] Fdn Policlin Tor Vergata, Dermatol Unit, I-00133 Rome, Italy

[2] Univ Roma Tor Vergata, Dept Syst Med, I-00133 Rome, Italy

来源：

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES | 2024年 / 25卷 / 13期

关键词：

blastic plasmacytoid dendritic cell neoplasm; BPDCN; skin cancer; cutaneous malignancies; INTERFERON-PRODUCING CELLS; T-CELLS; PREDENDRITIC CELLS; LANGERHANS CELLS; LYMPH-NODES; SKIN; TRANSPLANTATION; FEATURES; PROGENITORS; RECRUITMENT;

D O I：

10.3390/ijms25137099

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive hematological malignancy derived from the precursors of plasmacytoid dendritic cells. Although disease awareness has increased over time, BPDCN represents a rare disease with an aggressive clinical course and a dismal prognosis. Due to the overlap in clinical and histological features with a large spectrum of inflammatory and neoplastic diseases, BPDCN is difficult to diagnose. Furthermore, given the rarity of the disease, treatment options for BPDCN are limited, sometimes changing by practitioner and hospitals. Treatment options range from conventional chemotherapy to the recently approved biologic agent tagraxofusp and stem cell transplantation. Therefore, a multidisciplinary approach with coordination among dermatologists, pathologists, and hematologists is ultimately imperative to reach the correct diagnosis and management of BPDCN.

引用

页数：14

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