M-C Brüggen1,2,3, J Valencak4, R Stranzenbach5, N Li1,2, R Stadler5, C Jonak4, W Bauer4, S Porkert4, A Blaschke4, F Meiss6, J P Nicolay7, U Wehkamp8, M Schlaak9,10, V A Nguyen11, N Romani11, A Cozzio12, N Gayathri13, F Dimitriou1,2, L E French10, R Dummer1,2, E Guenova1,2,14. 1. Department of Dermatology, University Hospital of Zurich, Zurich, Switzerland. 2. Faculty of Medicine, University of Zurich, Zurich, Switzerland. 3. Medical Campus Davos, Davos, Switzerland. 4. Department of Dermatology, Medical University of Vienna, Vienna, Austria. 5. Department of Dermatology, Venereology, Allergology and Phlebology, Johannes Wesling Medical Centre, University Hospital of Ruhr-University Bochum, Minden, Germany. 6. Department of Dermatology and Venereology, Faculty of Medicine, University Medical Center - University of Freiburg, Freiburg, Germany. 7. Department of Dermatology, Venereology and Allergology, University Medical Centre Mannheim, University of Heidelberg, Mannheim, Germany. 8. Department of Dermatology, University Hospital of Schleswig-Holstein, Kiel, Germany. 9. Department of Dermatology and Venereology, University Hospital of Cologne, Cologne, Germany. 10. Department of Dermatology and Allergology, Ludwig-Maximilians-University of Munich, Munich, Germany. 11. Department of Dermatology, Venereology and Allergology, Medical University of Innsbruck, Innsbruck, Austria. 12. Department of Dermatology, Venereology and Allergology, Canton Hospital of St. Gallen, St. Gallen, Switzerland. 13. Department for Hematology, University Hospital of Zurich, Zurich, Switzerland. 14. Department of Dermatology, University Hospital Lausanne and University of Lausanne, Lausanne, Switzerland.
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.
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.
Authors: Anna Maria Florescu; Anne Louise Tølbøll Sørensen; Henrik Vedel Nielsen; Daniel Tolnai; Lene Dissing Sjö; Katja Lohmann Larsen; Mohammad Al-Mahdi Al-Karagholi Journal: BMC Neurol Date: 2022-06-24 Impact factor: 2.903
Authors: Kinan M Hayani; Gabriele Escherich; Karoline Koch; Lars E French; Hans H Wolff Journal: Acta Derm Venereol Date: 2020-08-18 Impact factor: 3.875