Publication

Clinical diversity and treatment approaches to blastic plasmacytoid dendritic cell neoplasm: a retrospective multicentre study

Journal Paper/Review - Jan 19, 2020

Units
PubMed
Doi

Citation
Brüggen M, Schlaak M, Nguyen V, Romani N, Cozzio A, Nair G, Dimitriou F, French L, Dummer R, Guenova E, Wehkamp U, Nicolay J, Valencak J, Stranzenbach R, Li N, Stadler R, Jonak C, Bauer W, Porkert S, Blaschke A, Meiß F, working group for cutaneous lymphoma of the Arbeitsgemeinschaft Dermatologische Forschung (ADF). Clinical diversity and treatment approaches to blastic plasmacytoid dendritic cell neoplasm: a retrospective multicentre study. J Eur Acad Dermatol Venereol 2020
Type
Journal Paper/Review (English)
Journal
J Eur Acad Dermatol Venereol 2020
Publication Date
Jan 19, 2020
Issn Electronic
1468-3083
Brief description/objective

BACKGROUND
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive type of hematologic precursor malignancy primarily often manifesting in the skin. We sought to provide a thorough clinical characterisation and report our experience on therapeutic approaches to BPDCN.

METHODS
In the present multicentric retrospective study, we collected all BPDCN cases occurring between 05/1999 and 03/2018 in 10 secondary care centres of the German-Swiss-Austrian cutaneous lymphoma working group.

RESULTS
A total of 37 BPDCN cases were identified and included. Almost 90% of the patients had systemic manifestations (bone marrow, lymph nodes, peripheral blood) in addition to skin involvement. The latter presented with various types of cutaneous lesions: nodular (in more than 2/3) and bruise-like (in 1/3) skin lesions, but also maculopapular exanthema (in circa 1/6). Therapeutically, 22 patients received diverse combinations of chemotherapeutic regimens and/or radiotherapy. Despite initial responses, all of them ultimately relapsed and died from progressive disease. Eleven patients underwent hematopoietic stem cell transplantation (HSCT; autologous HSCT n=3, allo-HSCT n=8). The mortality rate among HSCT patients was only 33.33% with a median survival time of 60.5 months.

CONCLUSION
Our study demonstrates the clinical diversity of cutaneous BPDCN manifestations and the positive development observed after the introduction of HSCT.