Blastic plasmacytoid dendritic cell neoplasm: First
retrospective study in the Czech Republic

J 2020

Blastic plasmacytoid dendritic cell neoplasm: First retrospective study in the Czech Republic

CERNAN, M., T. SZOTKOWSKI, M. HISEMOVA, P. CETKOVSKY, L. SRAMKOVA et. al.

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

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30204 Oncology

Country of publisher

Slovakia

Confidentiality degree

není předmětem státního či obchodního tajemství

References:

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

Abstract

V originále

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.

Citovat

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.

@article{1670349,
   author = {Cernan, M. and Szotkowski, T. and Hisemova, M. and Cetkovsky, P. and Sramkova, L. and Stary, J. and Ráčil, Zdeněk and Mayer, Jiří and Sramek, J. and Jindra, P. and Visek, B. and Zak, P. and Novak, J. and Kozak, T. and Furst, T. and Papajik, T.},
   article_location = {Bratislava},
   article_number = {3},
   doi = {http://dx.doi.org/10.4149/neo_2020_190507N407},
   keywords = {blastic plasmacytoid dendritic cell neoplasm; BPDCN; allogeneic hematopoietic cell transplantation; SL-401},
   language = {eng},
   issn = {0028-2685},
   journal = {Neoplasma},
   title = {Blastic plasmacytoid dendritic cell neoplasm: First retrospective study in the Czech Republic},
   url = {http://www.elis.sk/index.php?page=shop.product_details&flypage=flypage.tpl&product_id=6586&category_id=160&option=com_virtuemart&vmcchk=1&Itemid=1},
   volume = {67},
   year = {2020}
}

TY  - JOUR
ID  - 1670349
AU  - Cernan, M. - Szotkowski, T. - Hisemova, M. - Cetkovsky, P. - Sramkova, L. - Stary, J. - Ráčil, Zdeněk - Mayer, Jiří - Sramek, J. - Jindra, P. - Visek, B. - Zak, P. - Novak, J. - Kozak, T. - Furst, T. - Papajik, T.
PY  - 2020
TI  - Blastic plasmacytoid dendritic cell neoplasm: First retrospective study in the Czech Republic
JF  - Neoplasma
VL  - 67
IS  - 3
SP  - 650-659
EP  - 650-659
PB  - Slovenská akademie vied
SN  - 00282685
KW  - blastic plasmacytoid dendritic cell neoplasm
KW  - BPDCN
KW  - allogeneic hematopoietic cell transplantation
KW  - SL-401
UR  - http://www.elis.sk/index.php?page=shop.product_details&flypage=flypage.tpl&product_id=6586&category_id=160&option=com_virtuemart&vmcchk=1&Itemid=1
L2  - http://www.elis.sk/index.php?page=shop.product_details&flypage=flypage.tpl&product_id=6586&category_id=160&option=com_virtuemart&vmcchk=1&Itemid=1
N2  - 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.
ER  -

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. \textit{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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