CERNAN, M., T. SZOTKOWSKI, M. HISEMOVA, P. CETKOVSKY, L. SRAMKOVA, J. STARY, Zdeněk RÁČIL, Jiří MAYER, J. SRAMEK, P. JINDRA, B. VISEK, P. ZAK, J. NOVAK, T. KOZAK, T. FURST and T. PAPAJIK. Blastic plasmacytoid dendritic cell neoplasm: First retrospective study in the Czech Republic. Neoplasma. Bratislava: Slovenská akademie vied, 2020, vol. 67, No 3, p. 650-659. ISSN 0028-2685. Available from: https://dx.doi.org/10.4149/neo_2020_190507N407.
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Basic information
Original name Blastic plasmacytoid dendritic cell neoplasm: First retrospective study in the Czech Republic
Authors CERNAN, M. (203 Czech Republic), T. SZOTKOWSKI (203 Czech Republic, guarantor), M. HISEMOVA (203 Czech Republic), P. CETKOVSKY (203 Czech Republic), L. SRAMKOVA (203 Czech Republic), J. STARY (203 Czech Republic), Zdeněk RÁČIL (203 Czech Republic, belonging to the institution), Jiří MAYER (203 Czech Republic, belonging to the institution), J. SRAMEK (203 Czech Republic), P. JINDRA (203 Czech Republic), B. VISEK (203 Czech Republic), P. ZAK (203 Czech Republic), J. NOVAK (203 Czech Republic), T. KOZAK (203 Czech Republic), T. FURST (203 Czech Republic) and T. PAPAJIK (203 Czech Republic).
Edition Neoplasma, Bratislava, Slovenská akademie vied, 2020, 0028-2685.
Other information
Original language English
Type of outcome Article in a journal
Field of Study 30204 Oncology
Country of publisher Slovakia
Confidentiality degree is not subject to a state or trade secret
WWW URL
Impact factor Impact factor: 2.575
RIV identification code RIV/00216224:14110/20:00115982
Organization unit Faculty of Medicine
Doi http://dx.doi.org/10.4149/neo_2020_190507N407
UT WoS 000542668500022
Keywords in English blastic plasmacytoid dendritic cell neoplasm; BPDCN; allogeneic hematopoietic cell transplantation; SL-401
Tags 14110212, rivok
Tags International impact, Reviewed
Changed by Changed by: Mgr. Tereza Miškechová, učo 341652. Changed: 15/7/2020 09:23.
Abstract
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy with aggressive behavior and poor prognosis. We present the first retrospective analysis mapping its incidence and therapeutic outcomes in patients diagnosed and treated from 2000 to 2017 in the Czech Republic. The cohort comprised 14 patients (10 males, 4 females) with a median age at diagnosis of 39 years (range, 5-68 years). Initially, skin involvement was noted in 10 (71%) patients and bone marrow infiltration was present in 9 (64%). The first complete remission was achieved in 6/14 (43%) patients after acute lymphoblastic leukemia/lymphoma induction therapy and in 3/14 (21%) patients after acute myeloid leukemia regimen. Nine patients underwent allogeneic hematopoietic cell transplantation, with two patients achieving the first complete remission only after allogeneic transplantation. Patients undergoing allogeneic hematopoietic cell transplantation had longer overall survival than those treated without transplantation (the median survival over the period 16.4 vs. 8.1 months). Relapse of the disease was a significant predictor of mortality (p=0.05). Over the study period, patients' survival ranged from 3.3 to 44.2 months, with a median overall survival of 13 months. Our results revealed an effectivity of allogeneic hematopoietic cell transplantation on complete remission achievement in refractory/relapsed disease. The study aimed to present the actual data from the Czech Republic and thus contribute to a global understanding of BPDCN.
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