23 research outputs found

    Endobronhalnim ultrazvukom vođena aspiracija tankom iglom (EBUS-TBNA): Phyllodes tumor s me- tastazom u medijastinumu, vrlo rijedak tip metastatske neoplazme dojke

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    Phyllodes tumor of the breast, diagnosed predominantly in middle-aged women, is a rare fi- broepithelial neoplasm that makes up less than 1% of all breast neoplasms. Most phyllodes tumors have a benign nature with rare local recurrences, but some cytological subtypes are known for their malignant behavior. In this case report, we presented a patient with an extremely rare malignant sarcomatous differentiation of phyllodes tumor with metastasis to mediastinal lymph nodes, diagnosed by using endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA).Phyllodes (filoidni) tumor dojke, dijagnosticiran pretežno u žena srednje dobi, rijetka je fibroepitelna neo- plazma koja čini manje od 1% svih neoplazmi dojke. Većina filoidnih tumora dojke ima benignu prirodu s rijetkim lokalnim recidivima, ali određeni citoloÅ”ki podtipovi poznati su po svom malignom ponaÅ”anju. U ovom prikazu slučaja prikazali smo bolesnicu s iznimno rijetkom malignom sarkomatoznom diferencijacijom tumora filoidesa s metastazama u medijastinalne limfne čvorove, dijagnosticiranom uz pomoć transbronhalne aspiracije iglom vođene endobronhalnim ultrazvukom (engl. Endobronchial ultrasound-guided transbronchial needle aspiration, EBUS-TBNA)

    Different cytokine expression profiles in metaphyseal and diaphyseal fracture healing may provide new insights in the field of bone regeneration

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    Introduction Fractures are traumatic injuries that mainly occur in bone metaphysis, however most studies of bone healing have focused on diaphyseal bone. This is important because the healing process of trabecular metaphyseal bone has different healing characteristics from the diaphyseal area. Inflam- mation is thought to play an important, but different role in these two bone fracture types: diaphyseal fractures heal slowly through the formation of callus tissue, and metaphyseal trabecular bone heals faster, with no, or limited callus formation. As cytokines are key modulators of inflammation, the aim of the present study was to define the cytokine profiles at the core of these two conditions with possi- ble implications for the bone healing process. Materials and Methods This study included sixteen pa- tients with long bone metaphyseal and diaphyseal fractures and a healthy control group. Blood samples were taken at two timepoints: i) between the 1st (the day of the fracture) and the 6th day after fracture occurrence; ii) between the 7th and the 21st day after fracture occurrence. Fractures were treated either conservatively or surgically, depending on specific clinical indications. All participants with diaphyseal fractures were treated surgically. The control group provided blood samples on one occasion. The ob- tained plasma samples were pooled into 5 different experimental groups and analysed using commer- cial cytokine arrays. Results Marked differences in cytokine expression profiles were found between the fracture groups and the control group. The diaphyseal group had an ā€œactivatedā€ pro-inflammatory cytokine profile with markedly higher levels of cytokines at both timepoints compared to the meta- physeal group, which in contrast had a ā€œsilencedā€ cytokine expression profile. Single cytokine analysis revealed that in both metaphyseal and diaphyseal fracture groups MCP-1 and RANTES showed the most prominent fold change at both timepoints. IL-6 and TNF-Ī± also show similarly elevated levels in both timepoints in the diaphyseal fracture group, whereas this is not observed in the metaphyseal group. Furthermore, IL-3 expression was also elevated in the diaphyseal group, but only in the first timepoint. Conclusion This pilot study indicated chemokines which might be potential crucial driv- ers of bone healing, as well as painted distinct cytokine plasma profiles evident in metaphyseal and diaphyseal healing

    Maligna bolest, ili je neŔto drugo?

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    Sarcoidosis is a disease characterized by formation of granulomas in various tissues and organs. Changes most frequently occur in the lungs and lymph nodes.Sarkoidoza je bolest karakterizirana rastom granuloma u raznim tkivima i organima. Ove promjene se najčeŔće pojavljuju u plućima i limfnim čvorovima

    Different cytokine expression profiles in metaphyseal and diaphyseal fracture healing may provide new insights in the field of bone regeneration

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    Introduction Fractures are traumatic injuries that mainly occur in bone metaphysis, however most studies of bone healing have focused on diaphyseal bone. This is important because the healing process of trabecular metaphyseal bone has different healing characteristics from the diaphyseal area. Inflam- mation is thought to play an important, but different role in these two bone fracture types: diaphyseal fractures heal slowly through the formation of callus tissue, and metaphyseal trabecular bone heals faster, with no, or limited callus formation. As cytokines are key modulators of inflammation, the aim of the present study was to define the cytokine profiles at the core of these two conditions with possi- ble implications for the bone healing process. Materials and Methods This study included sixteen pa- tients with long bone metaphyseal and diaphyseal fractures and a healthy control group. Blood samples were taken at two timepoints: i) between the 1st (the day of the fracture) and the 6th day after fracture occurrence; ii) between the 7th and the 21st day after fracture occurrence. Fractures were treated either conservatively or surgically, depending on specific clinical indications. All participants with diaphyseal fractures were treated surgically. The control group provided blood samples on one occasion. The ob- tained plasma samples were pooled into 5 different experimental groups and analysed using commer- cial cytokine arrays. Results Marked differences in cytokine expression profiles were found between the fracture groups and the control group. The diaphyseal group had an ā€œactivatedā€ pro-inflammatory cytokine profile with markedly higher levels of cytokines at both timepoints compared to the meta- physeal group, which in contrast had a ā€œsilencedā€ cytokine expression profile. Single cytokine analysis revealed that in both metaphyseal and diaphyseal fracture groups MCP-1 and RANTES showed the most prominent fold change at both timepoints. IL-6 and TNF-Ī± also show similarly elevated levels in both timepoints in the diaphyseal fracture group, whereas this is not observed in the metaphyseal group. Furthermore, IL-3 expression was also elevated in the diaphyseal group, but only in the first timepoint. Conclusion This pilot study indicated chemokines which might be potential crucial driv- ers of bone healing, as well as painted distinct cytokine plasma profiles evident in metaphyseal and diaphyseal healing

    MAGNESIUM HOMEOSTASIS DISORDER IN PATIENTS WITH CHRONIC KIDNEY DISEASE

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    Magnezij je važan unutarstanični kation koji sudjeluje kao kofaktor u viÅ”e od Å”est stotina biokemijskih reakcija. Raspon koncentracije magnezija strogo je reguliran apsorpcijom u crijevu, izlučivanjem putem bubrega te puferiranjem u stanicama i koÅ”tanom tkivu zbog čega određivanje isključivo koncentracije magnezija u serumu često nije dostatno za cjelovitu procjenu razine magnezija u organizmu. Napredovanjem kronične bubrežne bolesti (KBB) dolazi do smanjenja glomerularne fi ltracije Å”to nerijetko dovodi do nastanka hipermagnezemije. Cilj ovog rada je povećati svijest o poremećaju homeostaze magnezija u bolesnika s KBB i mogućim posljedicama poremećaja njegove ravnoteže. Pri provođenju hemodijalize treba pažljivo odabrati koncentraciju magnezija u dijalizatu. KoriÅ”tenjem vode za dijalizu bez magnezija često dolazi do razvoja hipomagnezemije, a koriÅ”tenjem otopine s viÅ”im koncentracijama magnezija nuspojave su blaže. U bolesnika na dijalizi čeŔća je hipermagnezemija, dok hipomagnezemija najčeŔće nastaje zbog smanjene apsorpcije u jejunumu. Povezanost peritonejske dijalize i hipomagnezemije joÅ” nije dovoljno istražena. U bolesnika s transplantiranim bubregom hipomagnezemija je česta. Uočeno je viÅ”e mehanizama kojima niska koncentracija magnezija u serumu povisuje stopu smrtnosti u bolesnika s KBB-om; neki od njih su ubrzana kalcifi kacija krvnih žila, dijabetogeni učinak, poticanje razvoja dislipidemije te metaboličkog sindroma. Nadalje, teÅ”ka hipomagnezemija može izazvati nastanak smrtonosnih srčanih aritmija. Ne postoji usuglaÅ”eno miÅ”ljenje treba li se provoditi nadoknada magnezija u bolesnika s KBB-om, iako su neke studije pokazale da se na taj način mogu prevenirati dugoročne komplikacije i srčanožilni incidenti.Magnesium is an important intracellular cation that acts as a cofactor in over 600 biochemical reactions. Concentration range of magnesium is strictly regulated by intestinal absorption, renal excretion and via cellular and bone buffering; thus determining magnesium concentration in serum may not be suffi cient to fully assess magnesium levels in the body. Chronic kidney disease (CKD) progression leads to a decrease in glomerular fi ltration resulting in hypermagnesemia. The aim of this article is to increase the awareness of magnesium homeostasis disorders in CKD and possible repercussions of magnesium imbalance. Concentration of magnesium in dialysis fl uid should be determined very carefully during hemodialysis. Hypomagnesemia often occurs when dialysis fl uid without magnesium is used, while using dialysis fl uid with higher magnesium concentrations has been reported to have less side effects. Patients on dialysis often have hypermagnesemia, while hypomagnesemia is connected with lower absorption in jejunum. Connection between peritoneal dialysis and hypomagnesemia is not fully investigated. Hypomagnesemia is common in patients with kidney transplant. There are many mechanisms through which hypomagnesemia increases mortality rate in patients with CKD, including increased rate of blood vessel calcifi cation, pro-diabetic effects, increasing the risk of dyslipidemia and metabolic syndrome. Furthermore, severe hypomagnesemia can cause fatal heart arrhythmias. A consensus regarding magnesium supplementation in patients with CKD has not been reached, although some studies have shown that it might prevent longterm complications and cardiovascular incidents

    MAGNESIUM HOMEOSTASIS DISORDER IN PATIENTS WITH CHRONIC KIDNEY DISEASE

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    Magnezij je važan unutarstanični kation koji sudjeluje kao kofaktor u viÅ”e od Å”est stotina biokemijskih reakcija. Raspon koncentracije magnezija strogo je reguliran apsorpcijom u crijevu, izlučivanjem putem bubrega te puferiranjem u stanicama i koÅ”tanom tkivu zbog čega određivanje isključivo koncentracije magnezija u serumu često nije dostatno za cjelovitu procjenu razine magnezija u organizmu. Napredovanjem kronične bubrežne bolesti (KBB) dolazi do smanjenja glomerularne fi ltracije Å”to nerijetko dovodi do nastanka hipermagnezemije. Cilj ovog rada je povećati svijest o poremećaju homeostaze magnezija u bolesnika s KBB i mogućim posljedicama poremećaja njegove ravnoteže. Pri provođenju hemodijalize treba pažljivo odabrati koncentraciju magnezija u dijalizatu. KoriÅ”tenjem vode za dijalizu bez magnezija često dolazi do razvoja hipomagnezemije, a koriÅ”tenjem otopine s viÅ”im koncentracijama magnezija nuspojave su blaže. U bolesnika na dijalizi čeŔća je hipermagnezemija, dok hipomagnezemija najčeŔće nastaje zbog smanjene apsorpcije u jejunumu. Povezanost peritonejske dijalize i hipomagnezemije joÅ” nije dovoljno istražena. U bolesnika s transplantiranim bubregom hipomagnezemija je česta. Uočeno je viÅ”e mehanizama kojima niska koncentracija magnezija u serumu povisuje stopu smrtnosti u bolesnika s KBB-om; neki od njih su ubrzana kalcifi kacija krvnih žila, dijabetogeni učinak, poticanje razvoja dislipidemije te metaboličkog sindroma. Nadalje, teÅ”ka hipomagnezemija može izazvati nastanak smrtonosnih srčanih aritmija. Ne postoji usuglaÅ”eno miÅ”ljenje treba li se provoditi nadoknada magnezija u bolesnika s KBB-om, iako su neke studije pokazale da se na taj način mogu prevenirati dugoročne komplikacije i srčanožilni incidenti.Magnesium is an important intracellular cation that acts as a cofactor in over 600 biochemical reactions. Concentration range of magnesium is strictly regulated by intestinal absorption, renal excretion and via cellular and bone buffering; thus determining magnesium concentration in serum may not be suffi cient to fully assess magnesium levels in the body. Chronic kidney disease (CKD) progression leads to a decrease in glomerular fi ltration resulting in hypermagnesemia. The aim of this article is to increase the awareness of magnesium homeostasis disorders in CKD and possible repercussions of magnesium imbalance. Concentration of magnesium in dialysis fl uid should be determined very carefully during hemodialysis. Hypomagnesemia often occurs when dialysis fl uid without magnesium is used, while using dialysis fl uid with higher magnesium concentrations has been reported to have less side effects. Patients on dialysis often have hypermagnesemia, while hypomagnesemia is connected with lower absorption in jejunum. Connection between peritoneal dialysis and hypomagnesemia is not fully investigated. Hypomagnesemia is common in patients with kidney transplant. There are many mechanisms through which hypomagnesemia increases mortality rate in patients with CKD, including increased rate of blood vessel calcifi cation, pro-diabetic effects, increasing the risk of dyslipidemia and metabolic syndrome. Furthermore, severe hypomagnesemia can cause fatal heart arrhythmias. A consensus regarding magnesium supplementation in patients with CKD has not been reached, although some studies have shown that it might prevent longterm complications and cardiovascular incidents

    Characteristics of patients with acute ST-segment elevation myocardial infarction treated with different combinations of antiaggregation therapy: experience from the Croatian branch of the ISACS-CT Registry

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    Background and Aim: The relevance of dual antiplatelet therapy (DAPT) in acute ST-segment elevation myocardial infarction (STEMI) is well-established (aspirin and P2Y12 inhibitors).1 The role of glycoprotein (GP) IIb/IIIa inhibitors in clinical practice is not completely defined. Administration in the event of thrombotic complications is considered reasonable, although there is no evidence for routine use in primary percutaneous coronary intervention (pPCI). The aim was to analyze early outcomes of STEMI patients (pts) in the Croatian branch of the ISACS-CT (International Registry of Acute Coronary Syndromes in Transitional Countries) registry, depending on received antiaggregation therapy. Patients and Methods: Data were gathered retrospectively from pts hospitalized between January 2012 to October 2017. The study included 2503 pts with acute coronary syndrome, from which 48.9% (n=1224) were diagnosed with STEMI. The patients were divided into 4 groups depending on administered antiaggregation therapy. Results: For 7.8% (n=96) pts antiaggregation therapy data were missing, and 5.8% (n=71) were not treated with DAPT. Remaining 1057 (86.4%) pts were analyzed. Aspirin was administered in 95% of pts in the first 24 hours. 41.9% (n=443) of pts were additionally treated with clopidogrel, 16.1% (n=170) with ticagrelor, 28.6% (n=302) with clopidogrel and eptifibatide, and 13.4% (n=142) with ticagrelor and eptifibatide (Table 1). The groups did not differ in comorbidities, while pts receiving eptifibatide had lower systolic blood pressure on admission. Patients treated with eptifibatide were more frequently male, smokers, of younger age, had more thrombotic complications seen on coronary angiography (predominantly distal embolisation and ā€œno-reflowā€ phenomenon) and lower in-hospital mortality. In a multivariable regression model adjusted for age, gender, hypertension, diabetes, and pPCI, increasing age (OR=1.1), diabetes (OR=1.9) and pPCI (OR=0.5) remained relevant to in-hospital mortality. Conclusion: STEMI patients that are young, male and smokers are more frequently treated with eptifibatide, likely due to a higher burden of thrombotic complications. Unlike the choice of antiaggregation therapy, increasing age, diabetes and non-invasive management of STEMI were associated with in-hospital mortality

    Characterization of patients with myocardial infarction with non-obstructive coronary arteries ā€“ experience from the Croatian branch of the ISACS-CT Registry

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    Background and Aim: There are still wide knowledge gaps in myocardial infarction with non-obstructive coronary arteries (MINOCA) - a heterogeneous entity seen in 1-10% of patients with acute coronary syndrome (ACS)1. The aim is to determine characteristics of MINOCA in the Croatian branch of the ISACS-CT registry, and compare them to age- and gender-matched patients with unstable angina (UA), non-ST-segment elevation myocardial infarction (NSTEMI) and ST segment elevation myocardial infarction (STEMI). Patients and Methods: The study included 2487 patients with ACS. MINOCA was defined by simultaneous cardiac troponin levels >0.014 ng/L, symptoms of ischemia or significant ST-T changes in the ECG, and an absence of coronary artery stenosis of ā‰„ 50% on angiography. Age and gender-matching was performed from the remaining cohort by randomly sampling patients from the UA, STEMI and NSTEMI subgroups, based on the mean age Ā± 5 years and the gender ratio of the MINOCA group. Results: MINOCA was seen in 2.5% (n=63) of the cohort, initially categorized as UA (37%), NSTEMI (48%) and STEMI (16%). Median age was 62 (53, 71) years, 56% male. After age- and gender-matching, there were 36 UA (10% of the UA cohort), 135 NSTEMI (15%) and 198 STEMI (16%) patients in the ACS control subgroups. MINOCA patients had a lower prevalence of diabetes, hypertension, dyslipidemia, chronic kidney disease and tobacco use as compared to UA and NSTEMI. MINOCA patients used less antiplatelets, beta-blockers and statins before hospitalization. MINOCA and STEMI subgroups had a high incidence of chest pain symptoms and a short time from symptom onset to hospitalization. In the first 24h of hospitalization, less MINOCA patients were treated with antiplatelets and statins, and at discharge, they were less frequently prescribed with antiplatelet drugs compared to UA and NSTEMI (Table 1). Inhospital mortality was low, with no deaths in MINOCA and UA patients, and 2 and 3 deaths in NSTEMI and STEMI, respectively. Conclusion: MINOCA patients are mainly categorized as UA and NSTEMI at presentation, but have less comorbidities, more pronounced symptoms of typical chest pain, a shorter time from symptoms to hospitalization, lower levels of statin and antiaggregation prescription at admission, and antiaggregation at discharge. In-hospital mortality confirms MINOCA as low risk, however, long-term registry follow-up is needed to learn about longer term outcomes

    Autoimmune hemolytic anemias

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    UVOD: Autoimunosne hemolitičke anemije (AIHA) heterogena su skupina rijetkih bolesti koje se, ovisno o uzroku, mogu podijeliti na primarne ili sekundarne. Ovisno o vrsti podležećih protutijela, mogu se podijeliti na tople (wAIHA), hladne (cAIHA), mijeÅ”ane (mAIHA) ili paroksizmalnu hladnu hemoglobinuriju (PCH). ----- CILJ: Ispitati kliničke karakteristike odraslih novooboljelih bolesnika s AIHA-om te identificirati čimbenike koji utječu na tijek bolesti. ----- ISPITANICI I METODE: Unicentrična retrospektivna studija provedena u Kliničkom bolničkom centru Zagreb uključila je sve punoljetne novooboljele osobe s AIHA-om koje su imale pozitivan antiglobulinski test (DAT+), u razdoblju od 1. siječnja 2014. do 30. lipnja 2020. Osobe su praćene od dijagnoze do zaključno 26. travnja 2021. Analiza preživljenja, postizanje kompletne remisije (KR) te relapsa bolesti (Re) provedene su uklapanjem u Coxov model propocionalnih hazarda (kovarijance modela: koncentracija hemoglobina (Hb) pri dijagnozi (osim pri analizi Re), dob pri dijagnozi, spol, komorbiditeti (Charlsonin indeks komorbiditeta), etioloÅ”ka i seroloÅ”ka klasifikacija te terapija rituksimabom). Za pojedine kovarijance izračunat je omjer hazarda (HR). ----- REZULTATI: U navedenom razdoblju bilo je 71 novoobljelih, od kojih je praćenje u vremenu bilo moguće za njih 64; 51% novooboljelih bile su žene, a najčeŔći tipovi AIHA-e bili su sekundarna (83%) i wAIHA (73%). Većina bolesnika primala je kortikosteroidnu terapiju kao prvu liniju liječenja; rituksimab je primilo 22 (34%) bolesnika, u 13 (59%) bolesnika primijenjen u sklopu prve linije terapije (zajedno s kortikosteroidima). Medijan razdoblja praćenja iznosio je 2,11 (0,60 ā€“ 3,49) godina. Stopa smrtnosti iznosila je 19,1 na 100 osoba godina; a značajan utjecaj na smrtnost imali su dob (HR 1,047 (95% CI: 1,02-1,08), p=0,001), komorbiditeti (HR 1,28 (95% CI: 1,05-1,55), p=0,013) te mAIHA vs. wAIHA (HR 0,19 (95% CI: 0 ā€“ 0,68), p=0,011). Stopa incidencije KR iznosila je 9,2 na 10 osoba-godina, a značajan utjecaj na KR imali su ženski spol (HR 2,27 (95% CI: 1,01-4,92), p=0,037) i sekundarna AIHA (HR 0,34 (95% CI: 0-0,95), p=0,039). Stopa incidencije relapsa nakon postignute KR iznosila je 1,4 na 10 osoba-godina, a značajan utjecaj na relaps imali su mAIHA vs. wAIHA (HR 181,7 (95% CI: 3-11Ɨ103), p=0,038) te dob (HR 0,34 (95% CI: 0-0,96), p=0,04). ----- ZAKLJUČAK: Provedeno istraživanje prikazalo je kliničke karakteristike bolesnika AIHA-om te je kvantificiralo stopu smrtnosti i stope incidencije kompletne remisije i relapsa bolesti, a ujedno je identificiralo čimbenike koje utječu na pojedini promatrani ishod.INTRODUCTION: Autoimmune hemolytic anemia (AIHA) is a heterogenous group of rare disorders which, depending upon the underlying etiology, can be classified as primary or secondary. Depending upon the underlying type of autoantibodies, AIHAs can be classified as warm (wAIHA), cold (cAIHA), mixed (mAIHA) or as paroxysmal cold hemoglobinuria (PCH). ----- AIM: To investigate the clinical characteristics of adult patients with AIHA and to identify factors that influence the course of disease. ----- PARTICIPANTS AND METHODS: Non-concurrent unicentric cohort study was performed at the University Hospital Centre Zagreb, which included all adult patients with newly diagnosed AIHA which had a positive direct antiglobulin test (DAT+), in the period from 1 January 2014 to 30 June 2020. All participants were followed until death or until censored, with the end of the follow-up period on 26 April 2021. Survival, complete remission (CR) and disease relapse (Re) were analyzed by fitting the Cox proportional hazards model (covariances: hemoglobin (Hb) concentration at diagnosis (except for Re analysis), age at diagnosis, sex, Charlson comorbidity index, etiological and serological classification, as well as rituximab therapy). Hazard ratio (HR) was calculated for each covariate. ----- RESULTS: During the mentioned time period, 71 DAT+ AIHA patients were newly diagnosed. Follow-up was possible for 64 of them. 51% of newly diagnosed patients were women, and the most common types were secondary (83%) and wAIHA (73%). Most patients received corticosteroids as first line therapy; 34% received rituximab therapy, in 59% of whom it was applied as first line. Median time of follow up was 2,11 (0,60 ā€“ 3,49) years. Mortality rate was 19.1 per 100 person-years; significant factors for mortality were age (HR 1,047 (95% CI: 1.0-1.1), p=0.001), comorbidities (HR 1.28 (95% CI: 1.1-1.6), p=0.013) and mAIHA vs. wAIHA (HR 0,19 (95% CI: 0 ā€“ 0.7), p=0.011). The incidence rate for CR was 9.2 per 10 person-years; significant factors for CR were female sex (HR 2.27 (95% CI: 1.01-4.9), p=0.037) and secondary AIHA (HR 0.34 (95% CI: 0-0.95), p=0.039). The relapse rate (after CR was achieved) was 1.4 per 10 person-years; significant factors for relapse were mAIHA vs. wAIHA (HR za mAIHA 181.7 (95% CI: 3-11Ɨ103), p=0.038) and age (HR 0.34 (95% CI: 0-0.96), p=0.04). ----- CONCLUSION: This study presented the clinical characteristics of DAT+ AIHA patients and quantified mortality rate and incidence rates for complete remission and relapse, as well as identified factors that significantly impact each of the observed outcomes

    Autoimmune hemolytic anemias

    No full text
    UVOD: Autoimunosne hemolitičke anemije (AIHA) heterogena su skupina rijetkih bolesti koje se, ovisno o uzroku, mogu podijeliti na primarne ili sekundarne. Ovisno o vrsti podležećih protutijela, mogu se podijeliti na tople (wAIHA), hladne (cAIHA), mijeÅ”ane (mAIHA) ili paroksizmalnu hladnu hemoglobinuriju (PCH). ----- CILJ: Ispitati kliničke karakteristike odraslih novooboljelih bolesnika s AIHA-om te identificirati čimbenike koji utječu na tijek bolesti. ----- ISPITANICI I METODE: Unicentrična retrospektivna studija provedena u Kliničkom bolničkom centru Zagreb uključila je sve punoljetne novooboljele osobe s AIHA-om koje su imale pozitivan antiglobulinski test (DAT+), u razdoblju od 1. siječnja 2014. do 30. lipnja 2020. Osobe su praćene od dijagnoze do zaključno 26. travnja 2021. Analiza preživljenja, postizanje kompletne remisije (KR) te relapsa bolesti (Re) provedene su uklapanjem u Coxov model propocionalnih hazarda (kovarijance modela: koncentracija hemoglobina (Hb) pri dijagnozi (osim pri analizi Re), dob pri dijagnozi, spol, komorbiditeti (Charlsonin indeks komorbiditeta), etioloÅ”ka i seroloÅ”ka klasifikacija te terapija rituksimabom). Za pojedine kovarijance izračunat je omjer hazarda (HR). ----- REZULTATI: U navedenom razdoblju bilo je 71 novoobljelih, od kojih je praćenje u vremenu bilo moguće za njih 64; 51% novooboljelih bile su žene, a najčeŔći tipovi AIHA-e bili su sekundarna (83%) i wAIHA (73%). Većina bolesnika primala je kortikosteroidnu terapiju kao prvu liniju liječenja; rituksimab je primilo 22 (34%) bolesnika, u 13 (59%) bolesnika primijenjen u sklopu prve linije terapije (zajedno s kortikosteroidima). Medijan razdoblja praćenja iznosio je 2,11 (0,60 ā€“ 3,49) godina. Stopa smrtnosti iznosila je 19,1 na 100 osoba godina; a značajan utjecaj na smrtnost imali su dob (HR 1,047 (95% CI: 1,02-1,08), p=0,001), komorbiditeti (HR 1,28 (95% CI: 1,05-1,55), p=0,013) te mAIHA vs. wAIHA (HR 0,19 (95% CI: 0 ā€“ 0,68), p=0,011). Stopa incidencije KR iznosila je 9,2 na 10 osoba-godina, a značajan utjecaj na KR imali su ženski spol (HR 2,27 (95% CI: 1,01-4,92), p=0,037) i sekundarna AIHA (HR 0,34 (95% CI: 0-0,95), p=0,039). Stopa incidencije relapsa nakon postignute KR iznosila je 1,4 na 10 osoba-godina, a značajan utjecaj na relaps imali su mAIHA vs. wAIHA (HR 181,7 (95% CI: 3-11Ɨ103), p=0,038) te dob (HR 0,34 (95% CI: 0-0,96), p=0,04). ----- ZAKLJUČAK: Provedeno istraživanje prikazalo je kliničke karakteristike bolesnika AIHA-om te je kvantificiralo stopu smrtnosti i stope incidencije kompletne remisije i relapsa bolesti, a ujedno je identificiralo čimbenike koje utječu na pojedini promatrani ishod.INTRODUCTION: Autoimmune hemolytic anemia (AIHA) is a heterogenous group of rare disorders which, depending upon the underlying etiology, can be classified as primary or secondary. Depending upon the underlying type of autoantibodies, AIHAs can be classified as warm (wAIHA), cold (cAIHA), mixed (mAIHA) or as paroxysmal cold hemoglobinuria (PCH). ----- AIM: To investigate the clinical characteristics of adult patients with AIHA and to identify factors that influence the course of disease. ----- PARTICIPANTS AND METHODS: Non-concurrent unicentric cohort study was performed at the University Hospital Centre Zagreb, which included all adult patients with newly diagnosed AIHA which had a positive direct antiglobulin test (DAT+), in the period from 1 January 2014 to 30 June 2020. All participants were followed until death or until censored, with the end of the follow-up period on 26 April 2021. Survival, complete remission (CR) and disease relapse (Re) were analyzed by fitting the Cox proportional hazards model (covariances: hemoglobin (Hb) concentration at diagnosis (except for Re analysis), age at diagnosis, sex, Charlson comorbidity index, etiological and serological classification, as well as rituximab therapy). Hazard ratio (HR) was calculated for each covariate. ----- RESULTS: During the mentioned time period, 71 DAT+ AIHA patients were newly diagnosed. Follow-up was possible for 64 of them. 51% of newly diagnosed patients were women, and the most common types were secondary (83%) and wAIHA (73%). Most patients received corticosteroids as first line therapy; 34% received rituximab therapy, in 59% of whom it was applied as first line. Median time of follow up was 2,11 (0,60 ā€“ 3,49) years. Mortality rate was 19.1 per 100 person-years; significant factors for mortality were age (HR 1,047 (95% CI: 1.0-1.1), p=0.001), comorbidities (HR 1.28 (95% CI: 1.1-1.6), p=0.013) and mAIHA vs. wAIHA (HR 0,19 (95% CI: 0 ā€“ 0.7), p=0.011). The incidence rate for CR was 9.2 per 10 person-years; significant factors for CR were female sex (HR 2.27 (95% CI: 1.01-4.9), p=0.037) and secondary AIHA (HR 0.34 (95% CI: 0-0.95), p=0.039). The relapse rate (after CR was achieved) was 1.4 per 10 person-years; significant factors for relapse were mAIHA vs. wAIHA (HR za mAIHA 181.7 (95% CI: 3-11Ɨ103), p=0.038) and age (HR 0.34 (95% CI: 0-0.96), p=0.04). ----- CONCLUSION: This study presented the clinical characteristics of DAT+ AIHA patients and quantified mortality rate and incidence rates for complete remission and relapse, as well as identified factors that significantly impact each of the observed outcomes
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