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1.
High burden of clonal mast cell disorders and hereditary ▫$α-tryptasemia$▫ in patients who need Hymenoptera venom immunotherapy
Peter Korošec, Gunter Sturm, Jonathan J. Lyons, Tinkara Pirc Marolt, Manca Svetina, Mitja Košnik, Mihaela Zidarn, Mark Kačar, Nina Frelih, Nika Lalek, Ajda Demšar Luzar, Samo Zver, Matevž Škerget, Ewa Czarnobilska, Wojciech Dyga, Sanja Popović-Grle, Miroslav Samaržija, Lisa Arzt-Gradwohl, Urban Čerpes, Grzegorz Porebski, Branko Pevec, Eva Schadelbauer, Peter Kopač, Julij Šelb, Matija Rijavec, 2024, original scientific article

Abstract: Background In patients who require venom immunotherapy (VIT), there is a need to identify underlying mast cell (MC) disorders since these may affect the risk and severity of future sting reactions and the long-term effectiveness of VIT. Methods 1319 individuals with Hymenoptera venom allergy (HVA) who needed VIT from referral centers in Slovenia, Austria, Croatia, and Poland underwent examination for KIT p.D816V in peripheral blood leukocytes (PBL) using a highly sensitive PCR test and tryptase genotyping by digital droplet PCR. We also included 183 control individuals with large local reactions (LLRs) to Hymenoptera stings and with asymptomatic sensitization to Hymenoptera venoms. Results 285 of 1319 individuals recommended for VIT (21.6%) were positive for KIT p.D816V in PBL, preferably those who present with severe reaction (33.9% [n = 207 of 610] with Ring-Messmer grade 3–4 vs. 11% [n = 78 of 709] with Grade 1–2; p < .0001), whereas only 1.3% (n = 2 of 152) of controls with LLR and none with asymptomatic sensitization (n = 31) had KIT p.D816V. KIT p.D816V allelic burden was higher in those with severe reaction (median 0.018% [n = 207] in Grade 3–4 vs. 0.001% [n = 78] in Grade 1–2; p < .0001), and the majority had normal baseline serum tryptase levels (69% [n = 196 of 285]). All KIT p.D816V-positive individuals (n = 41) who underwent bone marrow (BM) biopsy were found to have underlying clonal diseases, principally BM mastocytosis. HαT was also associated with severe HVA and symptoms (p < .01), and remarkably, 31.0% (n = 31 of 100) were found to have concomitant KIT p.D816V. Concomitant HαT and KIT p.D816V showed an additive effect, and having both was associated with the highest risk for severe HVA, even higher than having either HαT or KIT p.D816V alone (OR = 3.8; p < .01). Conclusions By employing prospective universal tryptase genotyping and examination for KIT p.D816V in PBL in large HVA populations, we have demonstrated a high burden of clonal MC disorders and HαT in patients who require VIT.
Keywords: anaphylaxis, hereditary α-tryptasemia, hypersensitivity, immunotherapy, mast cell, mastocytosis, venom
Published in DiRROS: 17.06.2024; Views: 137; Downloads: 79
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2.
Long term follow-up report of cardiac toxicity in patients with multiple myeloma treated with tandem autologous hematopoietic stem cell transplantation
Mirta Koželj, Samo Zver, Vesna Zadnik, 2013, original scientific article

Abstract: Background. Tandem autologous hematopoietic stem cell transplantation (ta-HSCT) is a standard treatment for multiple myeloma (MM). Patients receive a high-dose cyclophosphamide (CY), followed by two myeloablative cycles of melphalan (MEL). There are scarce data about long term cardiotoxicity. Patients and methods. We studied 12 patients (62.25 8.55 years) six years after the completion of MM treatment with ta-HCST. Late cardiotoxic effects were evaluated clinically and echocardiographically. Results. None of the patients developed clinical signs of heart failure, all were in sinus rhythm and NT-pro BNP concentration was elevated (778 902.76 pg/mL). The left ventricular (LV) size remained normal. The LV ejection fraction did not decrease (73.75 5.67%, 69.27 6.13%, p = NS). The LV diastolic function parameters (E, A, ratio E/A and A/a) did not change significantly. In tissue Doppler parameters we observed a nonsignificant decrease in Em (10.26 2.63 cm/s, 7.57 1.43 cm/s) and Sm velocities (8.7 0.87 cm/s, 7.14 1.17 cm/s, p = NS). The E/Em values were in an abnormal range (8.66 1.05, 10.55 2.03). Conclusions. The treatment of MM with ta-HSCT, during which patients receive a high dose CY followed by two myeloablative cycles of MEL, causes mild, chronic, partially reversible and clinically silent cardiotoxic side-effects. However, ta-HSCT in patients with MM is a safe regarding cardiotoxic side effects, but, because of increasing life expectancy needs long term attention.
Keywords: cardiotoxicity, echocardiography, myeloma
Published in DiRROS: 22.03.2024; Views: 274; Downloads: 71
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Akutna mieloična levkemija
Samo Zver, 2014, published professional conference contribution

Keywords: levkemije, mieloična levkemija
Published in DiRROS: 02.04.2020; Views: 2495; Downloads: 667
.pdf Full text (137,37 KB)

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Hodgkinov limfom : vodnik za bolnike
Barbara Jezeršek Novaković, Samo Zver, Metka Zajc, Aleksandra Grbič, Andreja Cirila Škufca Smrdel, Eda Vrtačnik-Bokal, Vesna Šalamun, Vida Stržinar, Lorna Zadravec-Zaletel, Matej Pečovnik, 2016, dictionary, encyclopaedia, lexicon, manual, atlas, map

Abstract: Z vodnikom želimo tako bolnike kot svojce čim bolje seznaniti z najnovejšimi spoznanji o Hodgkinovem limfomu, njegovem odkrivanju, razjasniti osnove bolezni, predstaviti načine zdravljenja, ukrepe ob neželenih stranskih učinkih zdravljenja s kemoterapijo, poskrbeti za dobro osveščenost o možnostih ohranjanja plodnosti in možnih poznih posledicah zdravljenja, približati zavedanje o pomenu psihološke podpore bolniku in njegovim svojcem ter ponuditi pomoč Združenja L&L. Z ozaveščenostjo in pridobljenim znanjem lahko bolnik pri zdravljenju bolezni še bolj dejavno sodeluje s svojim zdravnikom in s tem pomembno prispeva k doseganju končnega cilja vsakega bolnika – k popolni ozdravitvi limfoma.
Keywords: rak (medicina), krvotvorne matične celice, transplantacija, psihološka pomoč, plodnost, hormonsko nadomestno zdravljenje
Published in DiRROS: 12.09.2016; Views: 6998; Downloads: 1000
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