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A Journal on Dermatology and Sexually Transmitted Diseases

Official Journal of the Italian Society of Dermatology and Sexually Transmitted Diseases
Indexed/Abstracted in: EMBASE, PubMed/MEDLINE, Science Citation Index Expanded (SciSearch), Scopus
Impact Factor 1,014

Frequency: Bi-Monthly

ISSN 0392-0488

Online ISSN 1827-1820


Giornale Italiano di Dermatologia e Venereologia 2012 December;147(6):603-8


Blastic plasmacytoid dendritic cell neoplasm (BPDCN): the cutaneous sanctuary

Pileri A. 1, Delfino C. 2, Grandi V. 2, Agostinelli C. 3, Pileri S. A. 3, Pimpinelli N. 2

1 Division of Dermatology, Department of Internal Medicine, Geriatrics and Nephrology, University of Bologna, Bologna, Italy;
2 Section of Dermatology, Department of Critical Care, Medicine and Surgery, University of Florence Medical School, Florence, Italy;
3 Hematopathology Unit, Department of Hematology and Oncology “L. and A. Seragnoli”, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy

AIM:Blastic plasmacytoid dendritic cell neoplasm (BPDNC) is a rare tumour, which stems from plasmacytoid dendritic cells. Although the aetiology is still unclear, in the last few years various reports suggested a potential role of chromosomal aberrations in the oncogenesis. The disease is currently enclosed among “acute myeloid leukemia (AML) and related precursor neoplasms” in the last WHO classification. BPDCN has an aggressive course, however, it has been suggested that an exclusive cutaneous involvement at presentation is related to a better clinical outcome.
METHODS: We review the literature about BPDCN, and we present a series of 11 cases, all characterised by disease limited to the skin at presentation. Furthermore, we examined all cases of the last 10 years stored in the database of the multidisciplinary study group on cutaneous lymphomas of the University of Florence.
RESULTS: Basing on the clinical features, patient were classified into two groups: with a single-lesion or multiple eruptive-lesions presentation. The former were treated with radiotherapy (limited field, electron beam therapy). The latter were treated with different therapeutic options, depending on age and co-morbidities. All patients with a single lesion achieved complete response. Five of 6 patients with eruptive lesions achieved a clinical response (2 complete and 3 partial response). Notably, the progression free survival was higher in the single-lesion than in the eruptive-lesion group (23 vs. 9 months). However all patients relapsed and 8 of 11 died.
CONCLUSION:Although the small number of selected patients, we could speculate that the concept of “cutaneous sanctuary” is particularly true in patients with a single lesion-presentation. In these patients, especially if >70 year-old aged, radiotherapy should be encouraged as the treatment of choice.

language: English


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