Blastic plasmacytoid dendritic cell neoplasm: First retrospective study in the Czech Republic
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F61989592%3A15110%2F20%3A73603640" target="_blank" >RIV/61989592:15110/20:73603640 - isvavai.cz</a>
Alternative codes found
RIV/00216224:14110/20:00115982 RIV/00216208:11120/20:43920169 RIV/00216208:11130/20:10411229 RIV/65269705:_____/20:00072818 and 5 more
Result on the web
<a href="http://www.elis.sk/download_file.php?product_id=6586&session_id=uo53ka3g19la4n4mdik1e2cu74" target="_blank" >http://www.elis.sk/download_file.php?product_id=6586&session_id=uo53ka3g19la4n4mdik1e2cu74</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.4149/neo_2020_190507N407" target="_blank" >10.4149/neo_2020_190507N407</a>
Alternative languages
Result language
angličtina
Original language name
Blastic plasmacytoid dendritic cell neoplasm: First retrospective study in the Czech Republic
Original language description
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.
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
30205 - Hematology
Result continuities
Project
—
Continuities
S - Specificky vyzkum na vysokych skolach
Others
Publication year
2020
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
NEOPLASMA
ISSN
0028-2685
e-ISSN
—
Volume of the periodical
67
Issue of the periodical within the volume
3
Country of publishing house
SK - SLOVAKIA
Number of pages
10
Pages from-to
650-659
UT code for WoS article
000542668500022
EID of the result in the Scopus database
2-s2.0-85084930521