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Iskalni niz: "avtor" (Biljana Grčar-Kuzmanov) .

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Do double-expressor high-grade B-cell lymphomas really need intensified treatment : a report from the real-life series of high-grade B-cell lymphomas treated with different therapeutic protocols at the Institute of Oncology Ljubljana
Lučka Boltežar, Samo Rožman, Gorana Gašljević, Biljana Grčar-Kuzmanov, Barbara Jezeršek Novaković, 2024, izvirni znanstveni članek

Povzetek: High-grade B-cell lymphomas with MYC and BCL2 and/or BCL6 rearrangements are known for their aggressive clinical course and so are the ones with MYC and BCL2 protein overexpression. The optimal therapy for these lymphomas remains to be elucidated. A retrospective analysis of all diffuse large B-cell lymphomas and high-grade B-cell lymphomas with MYC and BCL2 and/or BCL6 rearrangements diagnosed between 2017 and 2021 at the Institute of Oncology Ljubljana, Slovenia, has been performed. Only patients with double-expressor lymphoma (DEL), double-hit lymphoma (DHL), or triple-hit lymphoma (THL) were included. Demographic and clinical parameters were assessed, as well as progression-free survival (PFS) and overall survival (OS). In total, 161 cases out of 309 (161/309; 52,1%) were classified as DEL. Sixteen patients had DHL, MYC/BCL2 rearrangement was observed in eleven patients, and MYC/BCL6 rearrangement was observed in five patients. Five patients were diagnosed with THL. Out of 154 patients (according to inclusion/exclusion criteria) included in further evaluation, one-hundred and thirty-five patients had double-expressor lymphoma (DEL), sixteen patients had DHL, and three patients had THL. In total, 169 patients were treated with R-CHOP, 10 with R-CHOP and intermediate-dose methotrexate, 19 with R-DA-EPOCH, and 16 with other regimens. The median follow-up was 22 months. The 5-year OS for the whole DEL group was 57.1% (95% CI 45.9–68.3%) and the 5-year PFS was 76.5% (95% CI 72.6–80.4%). The log-rank test disclosed no differences in survival between treatment groups (p = 0.712) while the high-risk international prognostic index (IPI) carried a significantly higher risk of death (HR 7.68, 95% CI 2.32–25.49, p = 0.001). The 5-year OS for DHL patients was 32.4% (95% CI 16.6–48.2%) while all three TH patients were deceased or lost to follow-up. Our analyses of real-life data disclose that the R-CHOP protocol with CNS prophylaxis is a successful and curative treatment for a substantial proportion of DEL patients.
Ključne besede: lymphoma, double-expressor lymphoma, oncology
Objavljeno v DiRROS: 18.04.2024; Ogledov: 184; Prenosov: 70
.pdf Celotno besedilo (1004,13 KB)
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Ali visoko maligni B-celični limfomi, dvojni ekspresorji, res potrebujejo intenzivnejšo kemoterapijo
Lučka Boltežar, Samo Rožman, Gorana Gašljević, Biljana Grčar-Kuzmanov, Barbara Jezeršek Novaković, 2023, objavljeni znanstveni prispevek na konferenci

Povzetek: Velikocelični B limfomi z BCL2 in BCL6 preureditvijo so imenovani "double-hit" oziroma "triplehit" limfomi, z imunohistokemično ekspresijo pa dvojni ekspresorji (DE) in vsi imajo slabšo prognozo od klasičnih velikoceličnih limfomov B. Opravljena je bila študija vseh bolnikov z visoko malignimi B limfomi in difuznim velikoceličnim limfomom B, zdravljenih na Onkološkem inštitutu Ljubljana med leti 2017 in 2021. Večina izmed 154 vključenih bolnikov je bila zdravljenih z R-CHOP terapijo (rituksimab, ciklofosfamid, doksorubicin, vinkristin in prednizon), mediani čas spremljanja bolnikov je bil 22 mesecev. 110 bolnikov (71,4 %) ni imelo ponovitve bolezni v opazovanem obdobju. V primerjavi štirih uporabljenih terapevtskih protokolov v skupini DE nismo našli razlik v preživetju (p < 0,712). Preživetje bolnikov z "double-hit" in "triple-hit" limfomi je bilo, pričakovano, slabo. Naši podatki kažejo, da je velik del bolnikov z DE, zdravljen z R-CHOP in standardno CŽS profilakso, tudi uspešno ozdravljen. Za potrditev naših izsledkov so potrebne večje randomizirane raziskave.
Ključne besede: limfomi, kemoterapija, onkološko zdravljenje
Objavljeno v DiRROS: 16.06.2023; Ogledov: 441; Prenosov: 174
.pdf Celotno besedilo (1,93 MB)
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BAP1-defficient breast cancer in a patient with BAP1 cancer syndrome
Ana Blatnik, Domen Ribnikar, Vita Šetrajčič Dragoš, Srdjan Novaković, Vida Stegel, Biljana Grčar-Kuzmanov, Nina Boc, Barbara Perić, Petra Škerl, Gašper Klančar, Mateja Krajc, 2022, izvirni znanstveni članek

Povzetek: BAP1 cancer syndrome is a rare and highly penetrant hereditary cancer predisposition. Uveal melanoma, mesothelioma, renal cell carcinoma (RCC) and cutaneous melanoma are considered BAP1 cancer syndrome core cancers, whereas association with breast cancer has previously been suggested but not confirmed so far. In view of BAP1 immunomodulatory functions, BAP1 alterations could prove useful as possible biomarkers of response to immunotherapy in patients with BAP1-associated cancers. We present a case of a patient with BAP1 cancer syndrome who developed a metastatic breast cancer with loss of BAP1 demonstrated on immunohistochemistry. She carried a germline BAP1 likely pathogenic variant (c.898_899delAG p.(Arg300Glyfs*6)). In addition, tumor tissue sequencing identified a concurrent somatic variant in BAP1 (partial deletion of exon 12) and a low tumor mutational burden. As her triple negative tumor was shown to be PD-L1 positive, the patient was treated with combination of atezolizumab and nab-paclitaxel. She had a complete and sustained response to immunotherapy even after discontinuation of nab-paclitaxel. This case strengthens the evidence for including breast cancer in the BAP1 cancer syndrome tumor spectrum with implications for future cancer prevention programs. It also indicates immune checkpoint inhibitors might prove to be an effective treatment for BAP1-deficient breast cancer.
Ključne besede: BAP1, breast cancer, hereditary cancer syndromes, immunotherapy
Objavljeno v DiRROS: 19.09.2022; Ogledov: 516; Prenosov: 203
.pdf Celotno besedilo (1,12 MB)

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