Universität zu Köln

Bruggen, M-C., Valencak, J., Stranzenbach, R., Li, N., Stadler, R., Jonak, C., Bauer, W., Porkert, S., Blaschke, A., Meiss, F., Nicolay, J. P., Wehkamp, U., Schlaak, M., Nguyen, V. A., Romani, N., Cozzio, A., Gayathri, N., Dimitriou, F., French, L. E., Dummer, R. and Guenova, E. (2020). Clinical diversity and treatment approaches to blastic plasmacytoid dendritic cell neoplasm: a retrospective multicentre study. J. Eur. Acad. Dermatol. Venereol., 34 (7). S. 1489 - 1496. HOBOKEN: WILEY. ISSN 1468-3083

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Abstract

Background Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive type of haematologic precursor malignancy primarily often manifesting in the skin. We sought to provide a thorough clinical characterization and report our experience on therapeutic approaches toBPDCN. Methods In the present multicentric retrospective study, we collected allBPDCNcases 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 37BPDCNcases 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 haematopoietic stem cell transplantation (HSCT; autologousHSCTn = 3, allo-HSCTn = 8). The mortality rate amongHSCTpatients was only 33.33% with a median survival time of 60.5 months. Conclusion Our study demonstrates the clinical diversity of cutaneousBPDCNmanifestations and the positive development observed after the introduction ofHSCT.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Bruggen, M-C. UNSPECIFIED UNSPECIFIED UNSPECIFIED Valencak, J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Stranzenbach, R. UNSPECIFIED UNSPECIFIED UNSPECIFIED Li, N. UNSPECIFIED UNSPECIFIED UNSPECIFIED Stadler, R. UNSPECIFIED UNSPECIFIED UNSPECIFIED Jonak, C. UNSPECIFIED UNSPECIFIED UNSPECIFIED Bauer, W. UNSPECIFIED UNSPECIFIED UNSPECIFIED Porkert, S. UNSPECIFIED UNSPECIFIED UNSPECIFIED Blaschke, A. UNSPECIFIED UNSPECIFIED UNSPECIFIED Meiss, F. UNSPECIFIED UNSPECIFIED UNSPECIFIED Nicolay, J. P. UNSPECIFIED UNSPECIFIED UNSPECIFIED Wehkamp, U. UNSPECIFIED UNSPECIFIED UNSPECIFIED Schlaak, M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Nguyen, V. A. UNSPECIFIED UNSPECIFIED UNSPECIFIED Romani, N. UNSPECIFIED UNSPECIFIED UNSPECIFIED Cozzio, A. UNSPECIFIED UNSPECIFIED UNSPECIFIED Gayathri, N. UNSPECIFIED UNSPECIFIED UNSPECIFIED Dimitriou, F. UNSPECIFIED UNSPECIFIED UNSPECIFIED French, L. E. UNSPECIFIED UNSPECIFIED UNSPECIFIED Dummer, R. UNSPECIFIED UNSPECIFIED UNSPECIFIED Guenova, E. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-327793
DOI:	10.1111/jdv.16215
Journal or Publication Title:	J. Eur. Acad. Dermatol. Venereol.
Volume:	34
Number:	7
Page Range:	S. 1489 - 1496
Date:	2020
Publisher:	WILEY
Place of Publication:	HOBOKEN
ISSN:	1468-3083
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language HEALTH-ORGANIZATION CLASSIFICATION; 2016 REVISION; TRANSPLANTATION; LEUKEMIA; DIAGNOSIS; FEATURES; LINEAGE; UPDATE; ENTITY Multiple languages Dermatology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/32779