30 research outputs found
Primarni limfomi srediÅ”njeg živÄanog sustava
Primary central nervous system lymphoma (PCNSL) is a distinct form of aggressive non-Hodgkinās lymphoma (NHL) confined to the central nervous system. PCNSL typically affects older population. Individuals with HIV infection are especially at risk of PCNSL development and their outcome is extremely poor. Due to the presence of the blood brain barrier, PCNSL is treated differently from other extranodal NHLs. The mainstay of treatment is the high-dose methotrexate (MTX). Despite the treatment, local relapses are frequent and almost inevitably fatal. Intensification of treatment is possible in patients younger than 60 years. Radiotherapy is effective but complicated with significant delayed neurotoxicity, especially in the elderly. There are no curative treatment options in older patients who represent the majority of patients. Novel less toxic agents have modest activity. Prospective multicentric trials are needed to establish the optimal treatment for PCNSL.Primarni limfom srediÅ”njeg živÄanog sustava (PLSZÅ ) je poseban oblik agresivnog ne-Hodgkinovog limfoma (NHL) lokaliziran u srediÅ”njem živÄanom sustavu. PLSZÅ tipiÄno zahvaÄa stariju populaciju. Za razvoj PLSZÅ -a posebno su riziÄne osobe s HIV infekcijom Äija je prognoza ekstremno loÅ”a. Zbog krvno-moždane barijere, PLSZÅ se lijeÄi drugaÄije od ostalih ekstranodalnih NHL. Temelj lijeÄenja su visoke doze metotreksata. UnatoÄ lijeÄenju, lokalni relapsi su Äesti i gotovo uvijek fatalni. Radioterapija je uÄinkovita, ali komplicirana znaÄajnom kasnom neurotoksiÄnosti, posebno u starijih. U starijih bolesnika, koji Äine veÄinu, nema terapijskih opcija koje bi dovele do izljeÄenja. Noviji, manje toksiÄni lijekovi, skromnog su djelovanja. Potrebne su prospektivne multicentriÄne studije kako bi se definiralo optimalno lijeÄenje PLSZÅ -a
From Polyuria to Pathological Fracture: A Challenging Case of Multiple Myeloma
Multiple myeloma is a proliferation of neoplastic plasma cells producing a monoclonal protein (M-protein). Multiple myeloma can present in various
ways with anemia, renal failure, hypercalcemia, and bone fractures as the most common signs
Causes of thrombocytopenia in COVID-19 patients
Trombocitopenija je Äesta u virusnim infekcijama, prisutna je u prosjeÄno 18 % oboljelih od bolesti COVID-19, a obiÄno je blaga (u rasponu od 100 do 150 Ć 10^9/L). Zastupljenija je meÄu teže oboljelima i predstavlja neovisan Äimbenik rizika za loÅ” ishod i mortalitet. Trombocitopenija je rezultat smanjene proizvodnje i poveÄane potroÅ”nje trombocita. Smanjenje proizvodnje trombocita može nastati zbog oÅ”teÄenja hematopoetskih progenitora te zbog oÅ”teÄenja kapilarne mreže pluÄa. PoveÄana je potroÅ”nja trombocita zbog njihove hiperaktivacije i trombotske mikroangiopatije kod kritiÄno bolesnih. U bolesti COVID-19 rjeÄe se može razviti i imuna trombocitopenija (ITP) te trombocitopenija uzrokovana razliÄitim lijekovima, ukljuÄujuÄi i trombocitopeniju uzrokovanu heparinom (HIT).Thrombocytopenia is frequent in viral infections. Approximately 18% of COVID-19 patients have thrombocytopenia that is usually mild (ranging from 100 to 150 Ć 10^9/L). It is more common among critically ill whereby it is an independent risk factor for poor outcome and mortality. Both decreased production and increased consumption of platelets may contribute. Decreased platelet production may occur due to a damage to hematopoietic progenitors and capillary network of the lungs. The consumption of platelets is increased due to their hyperactivation and thrombotic microangiopathy. Immune thrombocytopenia (ITP) and drug-induced thrombocytopenia including heparin-induced thrombocytopenia (HIT) can also develop during COVID-19 treatment
MetaboliÄni sindrom i ishod u bolesnika s akutnim infarktom miokarda
The impact of the metabolic syndrome/insulin resistance syndrome (MS/IR S) on the severity and prognosis of acute ST elevation myocardial infarction (STEMI ) treated with primary percutaneous coronary intervention (PCI) was assessed using the American Association of Clinical Endocrinologists and American College of Endocrinology (AACE/ACE) definition from 2003. A total of 385 patients having suffered acute STEMI and treated with primary PCI over a two-year period were divided into two groups (with and without MS/IR S) and compared according to the parameters of severity (clinical, laboratory, echocardiography, coronary angiography parameters and complications) and prognosis using major adverse cardiovascular events (MACE) during the six-month follow-up of acute STEMI . In comparison with control group, the MS/IR S group of patients had worse or similar results of almost all study parameters of severity (hospital days 6.5 vs. 6.5, cardiogenic shock 2.9% vs. 2.6%, cardiac arrest 6.8% vs. 5.2%, reinfarction 0.5 vs. 1.6%) and prognosis (total MACE 30.7 vs. 30.7%), however, none of the differences reached statistical significance. It is concluded that the unexpected lack of such differences in MS/IR S could be due to the absence of waist-to-hip ratio in the definition and other open questions in metabolic syndrome in general.Istraživao se utjecaj metaboliÄnog sindroma/sindroma inzulinske rezistencije (MS/SIR ) koristeÄi definiciju American Association of Clinical Endocrinologists i American College of Endocrinology (AACE/ACE) iz 2003. na težinu i prognozu akutnog infarkta miokarda s elevacijom ST spojnice (STEMI ) lijeÄenog primarnom perkutanom intervencijom (pPCI). Ukupno 395 bolesnika koji su preboljeli akutni STEMI i bili lijeÄeni pomoÄu pPCI u dvogodiÅ”njem razdoblju podijeljeno je u dvije skupine (s MS/SIR i bez njih) i usporeÄeno prema parametrima težine (kliniÄki, laboratorijski, ehokardiografski, koronarografski, komplikacije) i prognoze koristeÄi velike neželjene kardiovaskularne dogaÄaje (MACE) tijekom Å”estomjeseÄnog praÄenja akutnog STEMI . Skupina bolesnika s MS/SIR u usporedbi s kontrolnom skupinom imala je uglavnom loÅ”ije ili jednake rezultate težine (dani u bolnici 6,5:6,5, kardiogeni Å”ok 2,9%:2,6%, srÄani zastoj 6,8%:5,2%, reinfarkt 0,5%:1,6%) i prognoze (ukupno MACE 30,7%:30,7%), no niti jedna od razlika nije dosegla statistiÄku znaÄajnost. ZakljuÄuje se kako bi izostanak takvih oÄekivanih razlika u MS/SIR mogao biti posljedica iskljuÄenja omjera struka i kukova iz ove definicije i ostalih otvorenih pitanja u metaboliÄnom sindromu uopÄe
Biometrija sagitalnih otolita tri pridnene vrste riba istoÄnog Jadrana (Crna Gora)
The otolith morphology, biometry and otolith size and fish length relationship of three demersal fish species: red mullet Mullus barbatus, common pandora Pagellus erythrinus and hake Merluccius merluccius were examined. Based on otoliths relative sizes P. erythrinus and M. merluccius have Large otoliths, while M. barbatus has Small sagittal otoliths. The relationships between otolith variables and fish somatic growth were described by a linear function. All relationships were statistically highly significant (p<0.001). Otolith length (OL) was found to be the variable most strongly related to fish size, with 61.2 ā 95.3 % of the variability, while otolith weight had the lowest coefficient of determination (r2). No differences were found neither between left and right otoliths, nor between male and female otoliths in these relationships. For all species studied the relationships of otolith length, height and weight against total fish length were all found to have positive allometry, indicating that the accretion of otoliths is relatively faster than increase in fish body size. The shape indices of form factor (FF), roundness (RD), aspect ratio (AR), circularity (C), rectangularity (R) and ellipticity (E) were calculated and the relationships between otolith length (OL) and shape indices were determined.Ispitana je morfologija otolita, biometrija i odnos veliÄine otolita i duljine tri pridnene vrste riba: trlja Mullus barbatus, arbun Pagellus erythrinus i osliÄ Merluccius merluccius. Na temelju relativnih veliÄina otolita P. erythrinus i M. merluccius imaju velike otolite, dok M. barbatus ima male sagitalne otolite. Odnosi izmeÄu varijabli otolita i somatskog rasta riba opisani su linearnom funkcijom. Svi odnosi bili su statistiÄki visoko znaÄajni (p<0,001). UtvrÄeno je da je duljina otolita (OL) pokazuje najveÄu povezanost s veliÄinom ribe, sa 61,2 ā 95,3 % varijabilnosti, dok je masa otolita imala najniži koeficijent determinacije (r2). Nisu pronaÄene razlike ni izmeÄu lijevog i desnog otolita, niti izmeÄu muÅ”kih i ženskih otolita u tim odnosima. Za sve prouÄavane vrste utvrÄeno je da odnosi duljine, visine i mase otolita u odnosu na ukupnu duljinu ribe imaju pozitivnu alometriju, Å”to ukazuje da je nakupljanje otolita relativno brže od poveÄanja duljine tijela ribe. IzraÄunati su indeksi oblika faktora forme (FF), zaobljenosti (RD), omjera stranica (AR), kružnosti (C), pravokutnosti (R) i eliptiÄnosti (E) te su izraÄunati odnosi izmeÄu duljine otolita (OL) i indeksa oblika
Hereditary haemorrhagic telangiectasia ā systematic review and single center experience
Cilj istraživanja: Cilj je ovog rada prikazati kliniÄka iskustva naÅ”eg centra s oboljelima od hereditarne hemoragijske teleangiektazije (HHT). Prikazali smo kliniÄku sliku svojih bolesnika, nove dijagnostiÄke i terapijske moguÄnosti, kao i svjetske smjernice u dijagnostici bolesti. Ispitanici i metode: U ovu retrospektivnu studiju ukljuÄeno je 13-ero bolesnika lijeÄenih u Klinici za unutarnje bolesti KB-a Dubrava u razdoblju od 2008. do 2017. godine. Rezultati: Bilo je 9 muÅ”karaca i 4 žene u dobi izmeÄu 45 i 80 godina (medijan 70 godina). Svih 13-ero
bolesnika prezentiralo se sideropeniÄnom anemijom i rekurentnim epistaksama koje su ujedno bile i najraniji znak bolesti. Dvanaestero bolesnika imalo je mukokutane teleangiektazije, a njih devetero gastrointestinalna krvarenja. U jednog bolesnika dokazana je pulmonalna, a kod joÅ” jednoga hepatalna arteriovenska malformacija. Nakon medijana praÄenja od 60 mjeseci devetero je bolesnika živo, a njih Äetvero preminulo je. ZakljuÄci: Prava dijagnoza bolesti u veÄine naÅ”ih bolesnika postavljena je tek u starijoj dobi iako su se simptomi javljali i puno prije. U Republici Hrvatskoj ne postoje toÄni podaci o broju oboljelih, kao ni smjernice za otkrivanje i lijeÄenje komplikacija
bolesti. Nužno je formiranje multidisciplinarnih timova radi unaprjeÄenja dijagnostike, ranijeg otkrivanja komplikacija bolesti te sveobuhvatnijeg praÄenja bolesnika.Aim: The aim of this study is to present our centerās clinical experience with hereditary haemorrhagic telangiectasia (HHT). Herein, we showed clinical presentations of our patients, new diagnostic and therapeutic
options, as well as the international guidelines for HHT diagnosis. Patients and methods: In this retrospective study, we identified 13 patients that were treated at the Department of Medicine, Clinical Hospital Dubrava from 2008 to 2017. Results: There were nine men and four women aged between 45 and 80 years (median 70 years).
All 13 patients presented with sideropenic anaemia and recurrent epistaxis that was the earliest sign of the disease. Twelve patients had mucocutaneous telangiectasia, and nine patients were presented with gastrointestinal bleeding. One patient was diagnosed with pulmonary arteriovenous malformation, and the other one with hepatic malformation. After a median follow-up of 60 months, nine patients are alive, and four patients died. Conclusion: Most of our patients were diagnosed in older age, although the symptoms appeared much earlier in life. There are no data on the number of patients with HHT in the Republic of Croatia. Also, guidelines for diagnosis and screening for complications are lacking. Therefore, it is necessary to form a multidisciplinary team for diagnostic improvement, early detection of complications, and more intensive monitoring of patients
The degree of anisocytosis predicts survival in patients with primary myelofibrosis
Introduction: Red cell distribution width (RDW) provides a quantitative measure of anisocytosis and it is associated with the presence of subclinical systemic inflammation and a poor outcome in a variety of diseases when elevated. Anisocytosis is a feature of primary myelofibrosis (PMF) but itās prognostic role in PMF has not yet been evaluated. ----- Patients and methods: 33 newly-diagnosed patients with PMF were analyzed. Baseline RDW values were obtained in addition to CRP, LDH, complete blood count, iron metabolism parameters and JAK2 V617F mutational status. Patients were staged according to IPSS prognostic scoring system, liver and spleen size were assessed by palpation. ----- Results: Median RDW was 19.0% (15.2%-22.5%). RDW correlated significantly with hemoglobin level (p=0.005), CRP (p=0.031), spleen size (p=0.036) and IPSS score (p=0.003). Patients with more pronounced anisocytosis had an inferior overall survival (OS) ā very-high RDW (ā„19.0%) vs. high RDW (15.1%-18.9%) subgroup, HR 5.37, p=0.002. RDW remained significantly associated with OS (p=0.002) in a multivariate model including IPSS score, hemoglobin level and CRP. ----- Conclusion: A higher degree of anisocytosis is associated with more advanced disease features and a decreased overall survival. RDW encompasses standard prognostic score and may help in the rapid detection of patients with an unfavorable prognosis
Hemochromatosis gene mutations may affect the survival of patients with myelodysplastic syndrome
OBJECTIVES:
The recent availability of potent oral iron chelators is renewing an interest in the assessment of the possible impact of HFE genetics in MDS. -----
METHODS:
Thirty six newly diagnosed patients with MDS were studied for parameters of iron metabolism in addition to C282Y and H63D mutations of the HFE gene. -----
RESULTS:
Mutations were present in 11 out of 36 patients (31%), which were not different from our general population and were equally distributed among MDS subtypes. Mutated patients had higher ferritin levels (Pā=ā0.039) and lower TIBC (Pā=ā0.018). Ferritin was found to be higher for the untransfused mutated patients (Pā=ā0.017), but not for transfusion-dependent patients in whom ferritin levels correlated significantly with the number of blood units received (Pā=ā0.04). There was no difference in the number of blood units received between the mutated and wild type patients. A new observation made was that the mutated patients had a lower overall survival in addition to a poorer leukemia free survival (LFS) (Pā=ā0.004 and Pā=ā0.003, respectively). -----
DISCUSSION:
The HFE gene mutations are not more frequent in MDS patients. Iron overload in mutated patients was higher but there was no correlation found using supportive therapy for anemia. The effect of mutations on survival could be mediated by changes in iron metabolism. -----
CONCLUSION:
The HFE genotype may predict MDS prognosis and there is a need for further studies. It remains a challenging question if HFE mutated MDS patients should be considered for potent iron chelation therapy
High-dose ifosfamide and mitoxantrone (HDIM) in patients with relapsed or refractory Hodgkin's lymphoma
Relapsed/refractory Hodgkin's lymphoma (HL) is treated with salvage chemotherapy and autologous stem cell transplantation (ASCT). Optimal chemotherapy is unknown. We retrospectively analyzed outcomes of 58 patients treated with 2 cycles of high-dose ifosfamide and mitoxantrone (HDIM). HDIM consisted of ifosfamide 5 g/m(2)/day and MESNA 5 g/m(2)/day in continuous 24-h infusion (days 1 and 2), MESNA 2.5 g/m(2) over 12 h (day 3), and mitoxantrone 20 mg/m(2) (day 1) administered every 2 weeks. Stem cells were collected after the first cycle. Responding patients proceeded to ASCT. Toxicity was acceptable. Stem cell mobilization was successful in 96 % of patients. Overall response rate was 74 % (89 % in relapsing and 45 % in refractory patients) with 31 % complete remissions. After a median follow-up of 54 months, 5-year event-free survival was 56 % (69 % for relapsing and 35 % for refractory patients), and 5-year overall survival was 67 % (73 % for relapsing and 55 % for refractory patients). Significant adverse prognostic factors were refractoriness to previous therapy and HDIM failure. No differences in outcomes were noted between patients with early and late relapses or between complete and partial responders. HDIM is a well-tolerated and effective regimen for relapsed and refractory HL with excellent stem cell mobilizing properties. Patients failing HDIM may still benefit from other salvage options