Blastic plasmacytoid dendritic cell neoplasms: results of an international survey on 398 adult patients
| Autor: | Tomohiro Aoki, Mohamad Sobh, Alix Baugier de Materre, Carlo Cota, Kamel Laribi, Arthur E. Frankel, Caterina Giovanna Valentini, David Ghez, Ritsuro Suzuki, T. Petrella, Livio Pagano, Ronan Le Calloch, Kengo Takeuchi, Lorenzo Cerroni |
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| Rok vydání: | 2020 |
| Předmět: |
Adult
medicine.medical_specialty Palliative care Clinical Trials and Observations medicine.medical_treatment Plasmacytoid dendritic cell Hematopoietic stem cell transplantation Transplantation Autologous immune system diseases hemic and lymphatic diseases Internal medicine medicine Humans Chemotherapy Acute leukemia business.industry Hematopoietic Stem Cell Transplantation Dendritic Cells Hematology medicine.disease Lymphoma Radiation therapy Transplantation Leukemia Myeloid Acute Settore MED/15 - MALATTIE DEL SANGUE Acute Disease Blastic plasmocitoid dendritic cell leukemia business |
| Zdroj: | Blood Adv |
| ISSN: | 2473-9537 2473-9529 |
| DOI: | 10.1182/bloodadvances.2020002474 |
| Popis: | The purpose of this study is to describe the clinical and prognostic features and to evaluate the outcome of different therapeutic approaches among patients with blastic plasmacytoid dendritic cell neoplasm (BPDCN) who have been diagnosed and treated in different institutions. A total of 398 patients from 75 centers were included in the study. Treatment consisted of non-Hodgkin lymphoma (NHL)–like regimens in 129 (32.8%) patients and acute leukemia (AL)–like regimens in 113 (23.5%) patients. In 61 (15.5%) and 16 (4.1%) patients, chemotherapy was followed by allogeneic and autologous hematopoietic stem cell transplantation (HSCT), respectively. Twenty-seven (6.9%) patients received radiotherapy, 6 (1.5%) received new agents, and 62 (15.7%) received palliative care. After a median follow-up of 12 months, median overall survival (OS) was 18 months. Patients who received NHL/AL-like regimens, followed by allogeneic HSCT, had the best outcome; median OS was not reached. OS was 65 months for patients who underwent autologous HSCT; 18 months and 14 months, respectively, for those treated with AL-like and NHL-like regimens without consolidation; and 4 months for those receiving palliative care (P < .001). In BPDCN, chemotherapy with lymphoma- or AL-like regimens, followed by transplantation, represents the therapeutic strategy associated with the best outcome. Consolidation with allogeneic HSCT, when feasible, appears superior to autologous HSCT. |
| Databáze: | OpenAIRE |
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