Clinical diversity and treatment approaches to blastic plasmacytoid dendritic cell neoplasm: a retrospective multicentre study. (10th March 2020)

• Record Type: Journal Article
• Authors: Brüggen, 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.; Guenova, E.
• Is Part Of: Journal of the European Academy of Dermatology and Venereology. Volume 34:Number 7(2020)
• Page Start: 1489
• Page End: 1495
• Publication Date: 2020-03-10
• Subjects: Dermatology -- Periodicals; Sexually transmitted diseases -- Periodicals; 616.5
• DOI: 10.1111/jdv.16215
• Languages: English
• ISSNs: 0926-9959

Abstract

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 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.