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


Brüggen, Marie-Charlotte; Valencak, Julia; Stranzenbach, René; Li, Nick; Stadler, Rudolf; Jonak, Constanze; Bauer, Wolfgang; Porkert, Stefanie; Blaschke, Amelie; Meiss, Frank; Nicolay, Jan P; Wehkamp, Ulrike; Schlaak, Max; Nguyen, Van Anh; Romani, Nikolaus; Cozzio, Antonio; Gayathri, Nair; Dimitriou, Florentia; French, Lars E; Dummer, Reinhard; Guenova, Emmanuella (2020). Clinical diversity and treatment approaches to blastic plasmacytoid dendritic cell neoplasm: a retrospective multicentre study. Journal of the European Academy of Dermatology and Venerology, 34(7):1489-1495.

Abstract

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.

Abstract

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.

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Additional indexing

Contributors:working group for cutaneous lymphoma of the Arbeitsgemeinschaft Dermatologische Forschung (ADF)
Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Dermatology Clinic
Dewey Decimal Classification:610 Medicine & health
Scopus Subject Areas:Health Sciences > Dermatology
Health Sciences > Infectious Diseases
Language:English
Date:1 July 2020
Deposited On:21 Feb 2020 10:11
Last Modified:29 Jul 2020 14:07
Publisher:Wiley-Blackwell Publishing, Inc.
ISSN:0926-9959
OA Status:Closed
Publisher DOI:https://doi.org/10.1111/jdv.16215
PubMed ID:31955469

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