TY - JOUR
T1 - Blastic plasmacytoid dendritic cell neoplasms
T2 - Results of an international survey on 398 adult patients
AU - Laribi, Kamel
AU - De Materre, Alix Baugier
AU - Sobh, Mohamad
AU - Cerroni, Lorenzo
AU - Valentini, Caterina Giovanna
AU - Aoki, Tomohiro
AU - Suzuki, Ritsuro
AU - Takeuchi, Kengo
AU - Frankel, Arthur E.
AU - Cota, Carlo
AU - Ghez, David
AU - Le Calloch, Ronan
AU - Pagano, Livio
AU - Petrella, Tony
N1 - Publisher Copyright:
© 2020 by The American Society of Hematology.
PY - 2020/10
Y1 - 2020/10
N2 - 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 followup 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.
AB - 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 followup 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.
UR - http://www.scopus.com/inward/record.url?scp=85095881919&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85095881919&partnerID=8YFLogxK
U2 - 10.1182/BLOODADVANCES.2020002474
DO - 10.1182/BLOODADVANCES.2020002474
M3 - Article
C2 - 33027528
AN - SCOPUS:85095881919
SN - 2473-9529
VL - 4
SP - 4838
EP - 4848
JO - Blood Advances
JF - Blood Advances
IS - 19
ER -