Clinical diversity and treatment approaches to blastic plasmacytoid dendritic cell neoplasm : a retrospective multicentre study
© 2020 European Academy of Dermatology and Venereology..
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 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 haematopoietic 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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2020 |
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| Erschienen: |
2020 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:34 |
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| Enthalten in: |
Journal of the European Academy of Dermatology and Venereology : JEADV - 34(2020), 7 vom: 15. Juli, Seite 1489-1495 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Brüggen, M-C [VerfasserIn] |
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| Links: |
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| Anmerkungen: |
Date Completed 14.05.2021 Date Revised 14.05.2021 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1111/jdv.16215 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM305535641 |
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| 245 | 1 | 0 | |a Clinical diversity and treatment approaches to blastic plasmacytoid dendritic cell neoplasm |b a retrospective multicentre study |
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| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © 2020 European Academy of Dermatology and Venereology. | ||
| 520 | |a 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 to BPDCN | ||
| 520 | |a 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 | ||
| 520 | |a 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 haematopoietic 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 | ||
| 520 | |a CONCLUSION: Our study demonstrates the clinical diversity of cutaneous BPDCN manifestations and the positive development observed after the introduction of HSCT | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Multicenter Study | |
| 700 | 1 | |a Valencak, J |e verfasserin |4 aut | |
| 700 | 1 | |a Stranzenbach, R |e verfasserin |4 aut | |
| 700 | 1 | |a Li, N |e verfasserin |4 aut | |
| 700 | 1 | |a Stadler, R |e verfasserin |4 aut | |
| 700 | 1 | |a Jonak, C |e verfasserin |4 aut | |
| 700 | 1 | |a Bauer, W |e verfasserin |4 aut | |
| 700 | 1 | |a Porkert, S |e verfasserin |4 aut | |
| 700 | 1 | |a Blaschke, A |e verfasserin |4 aut | |
| 700 | 1 | |a Meiss, F |e verfasserin |4 aut | |
| 700 | 1 | |a Nicolay, J P |e verfasserin |4 aut | |
| 700 | 1 | |a Wehkamp, U |e verfasserin |4 aut | |
| 700 | 1 | |a Schlaak, M |e verfasserin |4 aut | |
| 700 | 1 | |a Nguyen, V A |e verfasserin |4 aut | |
| 700 | 1 | |a Romani, N |e verfasserin |4 aut | |
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| 700 | 1 | |a Guenova, E |e verfasserin |4 aut | |
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