Naslov (hrvatski) Sistemska mastocitoza u Republici Hrvatskoj Naslov (engleski) Systemic mastocytosis in Croatia Autor Ivan Krečak Autor Pavle Rončević Autor Marin Kursar Autor Marko Lucijanić Autor Davor Galušić Autor Stefan Mrđenović Autor Martina Morić Perić Autor Iva Ivanko Autor Hana Matijaca Autor Josipa Budimir Autor Sanja Jakelić Autor Ivana Karaman Autor Rajko Kušec Autorova ustanova Sveučilište u Zagrebu Znanstveno / umjetničko BIOMEDICINA I ZDRAVSTVO Sažetak (hrvatski) Cilj: Ciljevi ove studije bili su identificirati bolesnike sa sistemskom mastocitozom (SM) u Republici Hrvatskoj (RH) i analizirati njihove kliničke karakteristike. ----- Ispitanici i metode: Retrospektivno su iz osam hematoloških centara u RH identificirani bolesnici sa SM. Analizirane su kliničke karakteristike, te načini i ishodi liječenja ovih bolesnika. ----- Rezultati: Uključeno je 20 bolesnika, medijan dobi bio je 40,5 godina (raspon 24–77), a većinu su činile žene (n=12). Dominirali su bolesnici s indolentnom SM (ISM, n=11), dok je učestalost agresivne SM (ASM, n=4), „šuljajuće“ sistemske mastocitoze (SSM, n=3) i SM s pridruženom zloćudnom hematološkom bolešću (SM-AHND, n=2) bila manja. Gotovo su svi bolesnici imali kožni osip, a značajan broj njih i dispeptične smetnje, alergijsku dijatezu, bolove u kostima i osteoporozu. Antihistaminike je primala većina bolesnika, a citoredukciju 10 bolesnika (ISM=3, SSM=2, ASM=4, SM-AHND=1). Većina bolesnika koja je zahtijevala citoreduktivno liječenje primala je interferon alfa-2a (2
ISM, 1 SSM i 3 ASM), dva steroida (1 ISM i 1 SM-AHND), te po jedan imatinib (SSM) i kladribin (ASM). Svi su bolesnici liječeni u prvoj liniji interferonom alfa-2a i kladribinom postigli parcijalnu remisiju, a dva bolesnika liječena imatinibom i steroidom bila su refraktorna na liječenje. Nije bilo prekida liječenja interferonom zbog nuspojava. Nakon medijana praćenja od 33 mjeseca preminulo je troje bolesnika, jedan s ASM i oba s SM-AHND. Medijan preživljenja bolesnika s ISM/SSM nije dostignut naspram bolesnika s ASM/SM-AHND, gdje je iznosio 105 mjeseci (p=0,009). ----- Zaključak: Kliničke karakteristike i ishodi liječenja bolesnika sa SM u RH slični su onima iz velikih svjetskih centara. Najčešće korišten citoreduktivni lijek u RH bio je interferon alfa-2a koji se pokazao sigurnim i učinkovitim.
Sažetak (engleski) Aim: The aims of this study were to identify patients with systemic mastocytosis (SM) in Croatia and to analyze their clinical characteristics. ----- Patients and methods: Patients with SM treated at eight hospitals in Croatia were retrospectively identified and their clinical characteristics, treatment patterns and outcomes were analyzed. ----- Results:Twenty patients were included, median age was 40.5years (range 24-77), and most were females (n=12) . Patients with indolent SM (ISM, n=11) predominated, followed by aggressive SM (ASM, n=4), smoldering SM (SSM, n=3) and SM with an associated hematological neoplastic disorder (SM-AHND, n=2). Only one patient (with ASM) did not have cutaneous involvement, and a significant proportion of SM patients had dyspepsia, allergic diathesis, bone pains and osteoporosis. Antihistamines were administered in the majority of the patients, whereas ten patients needed cytoreductive treatment (ISM, n=3, SSM n=2, ASM, n=4, SM-AHND, n=1). Most SM patients in need for cytoreduction received interferon alpha-2a (two ISM, one SSM and three ASM), two received steroids (one ISM and one SM-AHND), one received imatinib (SSM) and the last patient was treated with cladribine (ASM). All patients treated first-line with interferons and cladribine achieved partial remission, whereas two patients treated with imatinib and steroid were refractory. None of the patients discontinued interferon due to drug-related side-effects. After a median follow-up of 33 months, three patients died, one with ASM and two with SM-AHND.The median survival of ISM/SSM patients was higher than in ASM/SM-AHND patients in whom it was 105 months (p=0.009). ----- Conclusion: Clinical characteristics and treatment outcomes of SM patients in Croatia are comparable to those from large international centers. The most commonly administered cytoreductive drug in Croatia was interferon alpha-2a which was shown to be safe and effective.
Ključne riječi (hrvatski)
SISTEMSKA MASTOCITOZA – dijagnoza, farmakoterapija, patologija
ANTIHISTAMINICI – terapijska uporaba
INTERFERON ALFA – terapijska uporaba
KLADRIBIN – terapijska uporaba
OSIP – etiologija
ISHOD LIJEČENJA
ANALIZA PREŽIVLJENJA
HRVATSKA
Ključne riječi (engleski)
MASTOCYTOSIS, SYSTEMIC – diagnosis, drug therapy, pathology
HISTAMINE ANTAGONISTS – therapeutic use
INTERFERON-ALPHA – therapeutic use
CLADRIBINE – therapeutic use
EXANTHEMA – etiologija
TREATMENT OUTCOME
SURVIVAL ANALYSIS
CROATIA
Jezik hrvatski Vrsta publikacije Znanstveni rad - Izvorni znanstveni rad Status objave Objavljen Vrsta recenzije Recenziran Verzija publikacije Objavljena verzija rada (izdavačev PDF) Naslov časopisa Liječnički vjesnik Brojčani podaci vol. 144, br. 9-10, str. 306-313 p-ISSN 0024-3477 e-ISSN 1849-2177 DOI https://doi.org/10.26800/LV-144-9-10-3 URN:NBN urn:nbn:hr:105:688284 Datum objave publikacije 2022 URL dokumenta https://hrcak.srce.hr/284416 Vrsta resursa Tekst Prava pristupa Otvoreni pristup Uvjeti korištenja Datum i vrijeme pohrane 2023-01-24 10:41:26
