Title (croatian) Sistemska mastocitoza u Republici Hrvatskoj Title (english) Systemic mastocytosis in Croatia Author Ivan Krečak Author Pavle Rončević Author Marin Kursar Author Marko Lucijanić Author Davor Galušić Author Stefan Mrđenović Author Martina Morić Perić Author Iva Ivanko Author Hana Matijaca Author Josipa Budimir Author Sanja Jakelić Author Ivana Karaman Author Rajko Kušec Author's institution University of Zagreb Scientific / art field, BIOMEDICINE AND HEALTHCARE Abstract (croatian) 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.
Abstract (english) 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.
Keywords (croatian)
SISTEMSKA MASTOCITOZA – dijagnoza, farmakoterapija, patologija
ANTIHISTAMINICI – terapijska uporaba
INTERFERON ALFA – terapijska uporaba
KLADRIBIN – terapijska uporaba
OSIP – etiologija
ISHOD LIJEČENJA
ANALIZA PREŽIVLJENJA
HRVATSKA
Keywords (english)
MASTOCYTOSIS, SYSTEMIC – diagnosis, drug therapy, pathology
HISTAMINE ANTAGONISTS – therapeutic use
INTERFERON-ALPHA – therapeutic use
CLADRIBINE – therapeutic use
EXANTHEMA – etiologija
TREATMENT OUTCOME
SURVIVAL ANALYSIS
CROATIA
Language croatian Publication type Scientific paper - Original scientific paper Publication status Published Peer review Peer review Publication version Published version Journal title Liječnički vjesnik Numbering vol. 144, no. 9-10, pp. 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 Publication 2022 Document URL https://hrcak.srce.hr/284416 Type of resource Text Access conditions Open access Terms of use Created on 2023-01-24 10:41:26
