-
Je něco špatně v tomto záznamu ?
Blastic plasmacytoid dendritic cell neoplasm: First retrospective study in the Czech Republic
M. Cernan, T. Szotkowski, M. Hisemova, P. Cetkovsky, L. Sramkova, J. Stary, Z. Racil, J. Mayer, J. Sramek, P. Jindra, B. Visek, P. Zak, J. Novak, T. Kozak, T. Furst, T. Papajik,
Jazyk angličtina Země Slovensko
Typ dokumentu časopisecké články
- MeSH
- dendritické buňky patologie MeSH
- dítě MeSH
- dospělí MeSH
- hematologické nádory mortalita terapie MeSH
- indukce remise MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- předškolní dítě MeSH
- recidiva MeSH
- retrospektivní studie MeSH
- senioři MeSH
- transplantace hematopoetických kmenových buněk * MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- předškolní dítě MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- Geografické názvy
- Česká republika MeSH
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.
Department of Hemato Oncology University Hospital Pilsen Pilsen Czech Republic
Institute of Hematology and Blood Transfusion Prague Czech Republic
Citace poskytuje Crossref.org
- 000
- 00000naa a2200000 a 4500
- 001
- bmc20025060
- 003
- CZ-PrNML
- 005
- 20220505113843.0
- 007
- ta
- 008
- 201125s2020 xo f 000 0|eng||
- 009
- AR
- 024 7_
- $a 10.4149/neo_2020_190507N407 $2 doi
- 035 __
- $a (PubMed)32064883
- 040 __
- $a ABA008 $b cze $d ABA008 $e AACR2
- 041 0_
- $a eng
- 044 __
- $a xo
- 100 1_
- $a Cernan, M $u Department of Hemato-Oncology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Olomouc, Czech Republic.
- 245 10
- $a Blastic plasmacytoid dendritic cell neoplasm: First retrospective study in the Czech Republic / $c M. Cernan, T. Szotkowski, M. Hisemova, P. Cetkovsky, L. Sramkova, J. Stary, Z. Racil, J. Mayer, J. Sramek, P. Jindra, B. Visek, P. Zak, J. Novak, T. Kozak, T. Furst, T. Papajik,
- 520 9_
- $a 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.
- 650 _2
- $a mladiství $7 D000293
- 650 _2
- $a dospělí $7 D000328
- 650 _2
- $a senioři $7 D000368
- 650 _2
- $a dítě $7 D002648
- 650 _2
- $a předškolní dítě $7 D002675
- 650 _2
- $a dendritické buňky $x patologie $7 D003713
- 650 _2
- $a ženské pohlaví $7 D005260
- 650 _2
- $a hematologické nádory $x mortalita $x terapie $7 D019337
- 650 12
- $a transplantace hematopoetických kmenových buněk $7 D018380
- 650 _2
- $a lidé $7 D006801
- 650 _2
- $a mužské pohlaví $7 D008297
- 650 _2
- $a lidé středního věku $7 D008875
- 650 _2
- $a recidiva $7 D012008
- 650 _2
- $a indukce remise $7 D012074
- 650 _2
- $a retrospektivní studie $7 D012189
- 650 _2
- $a mladý dospělý $7 D055815
- 651 _2
- $a Česká republika $7 D018153
- 655 _2
- $a časopisecké články $7 D016428
- 700 1_
- $a Szotkowski, T $u Department of Hemato-Oncology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Olomouc, Czech Republic.
- 700 1_
- $a Hisemová, Miloslava $u Institute of Hematology and Blood Transfusion, Prague, Czech Republic. $7 xx0272596
- 700 1_
- $a Cetkovsky, P $u Institute of Hematology and Blood Transfusion, Prague, Czech Republic.
- 700 1_
- $a Sramkova, L $u Department of Pediatric Hematology and Oncology, 2nd Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic.
- 700 1_
- $a Stary, J $u Department of Pediatric Hematology and Oncology, 2nd Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic.
- 700 1_
- $a Racil, Z $u Department of Internal Medicine, Hematology and Oncology, Masaryk University and University Hospital Brno, Brno, Czech Republic.
- 700 1_
- $a Mayer, J $u Department of Internal Medicine, Hematology and Oncology, Masaryk University and University Hospital Brno, Brno, Czech Republic.
- 700 1_
- $a Sramek, J $u Department of Hemato-Oncology, University Hospital Pilsen, Pilsen, Czech Republic.
- 700 1_
- $a Jindra, P $u Department of Hemato-Oncology, University Hospital Pilsen, Pilsen, Czech Republic.
- 700 1_
- $a Visek, B $u 4th Department of Internal Medicine - Hematology, University Hospital Hradec Kralove, Hradec Kralove, Czech Republic.
- 700 1_
- $a Zak, P $u 4th Department of Internal Medicine - Hematology, University Hospital Hradec Kralove, Hradec Kralove, Czech Republic.
- 700 1_
- $a Novak, J $u Department of Internal Medicine and Hematology, University Hospital Kralovske Vinohrady and Third Faculty of Medicine, Charles University, Prague, Czech Republic.
- 700 1_
- $a Kozak, T $u Department of Internal Medicine and Hematology, University Hospital Kralovske Vinohrady and Third Faculty of Medicine, Charles University, Prague, Czech Republic.
- 700 1_
- $a Furst, T $u Department of Mathematical Analysis and Application of Mathematics, Faculty of Science, Palacky University Olomouc, Olomouc, Czech Republic.
- 700 1_
- $a Papajik, T $u Department of Hemato-Oncology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Olomouc, Czech Republic.
- 773 0_
- $w MED00003470 $t Neoplasma $x 0028-2685 $g Roč. 67, č. 3 (2020), s. 650-659
- 856 41
- $u https://pubmed.ncbi.nlm.nih.gov/32064883 $y Pubmed
- 910 __
- $a ABA008 $b A 1194 $c 659 $y a $z 0
- 990 __
- $a 20201125 $b ABA008
- 991 __
- $a 20220505113837 $b ABA008
- 999 __
- $a ok $b bmc $g 1599205 $s 1115746
- BAS __
- $a 3
- BAS __
- $a PreBMC
- BMC __
- $a 2020 $b 67 $c 3 $d 650-659 $e 20200217 $i 0028-2685 $m Neoplasma $n Neoplasma $x MED00003470
- LZP __
- $a Pubmed-20201125