1. High burden of clonal mast cell disorders and hereditary ▫$α-tryptasemia$▫ in patients who need Hymenoptera venom immunotherapyPeter 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, izvirni znanstveni članek Povzetek: 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.
Ključne besede: anaphylaxis, hereditary α-tryptasemia, hypersensitivity, immunotherapy, mast cell, mastocytosis, venom
Objavljeno v DiRROS: 17.06.2024; Ogledov: 336; Prenosov: 236
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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 transplantationMirta Koželj, Samo Zver, Vesna Zadnik, 2013, izvirni znanstveni članek Povzetek: 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.
Ključne besede: cardiotoxicity, echocardiography, myeloma
Objavljeno v DiRROS: 22.03.2024; Ogledov: 405; Prenosov: 109
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3. Hodgkinov limfom : vodnik za bolnikeBarbara Jezeršek Novaković, Samo Zver, Metka Zajc, Aleksandra Grbič, Andreja Cirila Škufca Smrdel, Eda Vrtačnik-Bokal, Vesna Šalamun, Nina Kovačević, Lorna Zadravec-Zaletel, Matej Pečovnik, 2022, ni določena Ključne besede: rak (medicina), krvotvorne matične celice, transplantacija, psihološka pomoč, plodnost, hormonsko nadomestno zdravljenje
Objavljeno v DiRROS: 24.07.2023; Ogledov: 679; Prenosov: 201
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4. Severe cold urticaria can point to an underlying clonal mast cell disorderMojca Bizjak, Marcus Maurer, Mitja Košnik, Dorothea Terhorst, Samo Zver, Thomas Burmeister, Frank Siebenhaar, 2021, drugi znanstveni članki Ključne besede: urticaria, cold urticaria, wheals, clonal mast cell disorder
Objavljeno v DiRROS: 10.05.2021; Ogledov: 1383; Prenosov: 413
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5. Hodgkinov limfom : vodnik za bolnikeBarbara Jezeršek Novaković, Samo Zver, Metka Zajc, Aleksandra Grbič, Andreja Cirila Škufca Smrdel, Eda Vrtačnik-Bokal, Vesna Šalamun, Vida Stržinar, Lorna Zadravec-Zaletel, Matej Pečovnik, 2014, slovar, enciklopedija, leksikon, priročnik, atlas, zemljevid Ključne besede: limfomi, maligni limfomi, simptomi, diagnostika, zdravljenje, kemoterapija, stranski učinki, zdravstveni nasveti
Objavljeno v DiRROS: 04.06.2020; Ogledov: 2553; Prenosov: 754
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7. Preživetje bolnikov z rakom, zbolelih v letih 1991-2005 v SlovenijiMaja Primic-Žakelj, Vesna Zadnik, Tina Žagar, Branko Zakotnik, Franc Anderluh, Nikola Bešić, Peter Černelč, Tanja Čufer, Andrej Debeljak, Ibrahim Edhemović, Janez Eržen, Albert-Peter Fras, Eldar Gadžijev, Marko Hočevar, Janez Jazbec, Barbara Jezeršek Novaković, Andrej Kmetec, Elga Majdič, Uroš Mlakar, Mojca Modic, Irena Oblak, Janja Ocvirk, Mirko Omejc, Stanislav Repše, Boris Sedmak, Uroš Smrdel, Mihael Sok, Primož Strojan, Breda Škrbinc, Alojz Šmid, Marjetka Uršič-Vrščaj, Vaneja Velenik, Samo Zver, Matjaž Zwitter, Janez Žgajnar, Mirjana Žumer-Pregelj, 2009, strokovna monografija Ključne besede: onkologija, zdravstvena statistika
Objavljeno v DiRROS: 14.09.2018; Ogledov: 3652; Prenosov: 1079
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8. Hodgkinov limfom : vodnik za bolnikeBarbara 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, slovar, enciklopedija, leksikon, priročnik, atlas, zemljevid Povzetek: 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.
Ključne besede: rak (medicina), krvotvorne matične celice, transplantacija, psihološka pomoč, plodnost, hormonsko nadomestno zdravljenje
Objavljeno v DiRROS: 12.09.2016; Ogledov: 7229; Prenosov: 1057
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