TREATMENT OF SEPSIS IN NEUTROPENIC PATIENTS

Febrilna neutropenija je česta i potencijalno fatalna komplikacija intenzivne citostatske terapije zbog čega je glavni uzrok nerelapsnog mortaliteta hematoloških pacijenata. Važnu skupinu pacijenata čine oni koji su zbog osnovne bolesti ili ranijih linija liječenja pod rizikom za infekcije specifi čnim uzročnicima. Kako se u većine pacijenata s febrilnom neutropenijom može postaviti dijagnoza sepse, dijagnostičke i terapijske postupke treba primjenjivati kao da je pacijent u sepsi. Bitno je prepoznati visokorizične pacijente jer se incidencija sepse u neutropeničnih pacijenata može znatno smanjiti adekvatnom antimikrobnom profilaksom i granulocitnim faktorima rasta. Ako dođe do sepse, vrlo je važno što ranije započeti liječenje prema ustanovljenim smjernicama, ali uvijek uzimajući u obzir i individualne karakteristike svakog pacijenta kao i mikrobiološku situaciju, čime se može znatno smanjiti mortalitet i morbiditet ovih pacijenata.Febrile neutropenia is a common and potentially fatal complication of intense cytotoxic therapy, which makes it the main cause of non-relapse mortality in patients with hematologic malignancies. Some of the patients are at risk of specifi c infections due to underlying disease of previous treatment regimens. Considering that most febrile neutropenic patients can be diagnosed with sepsis, diagnosis and treatment should be treated as septic. It is important to recognize patients at high risk since the incidence of sepsis in neutropenic patients can be signifi cantly reduced using antimicrobial prophylaxis and granulocyte growth factors. Once sepsis occurs, prompt treatment according to guidelines, individualized based on the specifi c problems of each patient, and microbiological situation can significantly reduce mortality and morbidity

Mitral and tricuspid valvuloplasty in a patient with myelodysplastic syndrome

Mijelodisplastični sindrom (MDS) klonska je bolest matične hematopoetske stanice, koja se očituje poremećenom proliferacijom, diferencijacijom i sazrijevanjem hematopoeze te displastičnim promjenama u koštanoj srži. U kliničkoj slici i laboratorijskim nalazima bolesnika s MDS-om dominira anemija, nešto rjeđe neutropenija ili trombocitopenija različitih stupnjeva težine. Kardiokirurški zahvat u bolesnika s MDS-om velik je izazov jer su neutropenija i trombocitopenija vodeći uzroci infekcija i krvarenja tijekom operacije. Malen je broj izvješća u literaturi koja opisuju kardiokirurški zahvat u bolesnika s MDS-om. Prikazujemo 66-godišnju bolesnicu s MDS-om kod koje je uspješno izvedena rekonstrukcija mitralnog i trikuspidalnoga srčanog zalistka. Bolesnica se javila u hitnu službu zbog otežanog disanja, opće slabosti i znakova srčanog popuštanja. Radiološkom obradom verificirani su obostrani pleuralni izljev, izraženije lijevo, i povećana sjena srca s naglašenim vaskularnim hilusima. Daljnjom obradom dokazane su mitralna i trikuspidalna insuficijencija teškog stupnja pa je bolesnica operirana, a pet godina nakon zahvata ima dobru kvalitetu života. Multidisciplinarnom suradnjom kardiologa, hematologa, anesteziologa i kardiokirurga u bolesnice je uspješno izveden kirurški zahvat rekonstrukcije mitralne i trikuspidalne valvule. Ovaj prikaz bolesnice upućuje na važnost multidisciplinarnog pristupa specifičnoj i ranjivoj grupi bolesnika s MDS-om i kardiovaskularnim komorbiditetima.Myelodysplastic syndrome (MDS) is a clonal disease of mutated hematopoietic stem cells characterized by abnormal hematopoietic differentiation and dysplastic bone marrow changes. MDS usually tends to present with anemia, less often with neutropenia and thrombocytopenia of varying degrees. Cardiac surgery in MDS patients is a major challenge because neutropenia and thrombocytopenia are the leading causes of infection and bleeding during the procedure. There are only a few reports of cardiovascular surgery in patients with MDS. We are presenting a 66-year-old MDS patient with a successful reconstruction of the mitral and tricuspid heart valve. The patient presented in the Emergency department with weakness and dyspnea. Radiological workup verified 3rd stage heart failure. Severe mitral and tricuspid insufficiency was verified, after which the patient underwent cardiac surgery, and five years after the procedure has good quality of life. Through the multidisciplinary collaboration of cardiologists, hematologists and cardiac surgeons an operative reconstruction of mitral and tricuspid valve was successfully performed. This case indicates the need for multidisciplinary care for the specific and vulnerable group of MDS patients with cardiovascular comorbidities

Smjernice za dijagnozu i liječenje bolesnika s mijelodisplastičnim sindromom [Guidelines for diagnosis and treatment of patients with myelodysplastic syndrome]

Myelodysplastic syndrome Working Group of the Croatian Cooperative Group for Hematologic Diseases (CROHEM), Referral center of the Ministry of Health of the Republic of Croatia for diagnostics and treatment of MDS, as well as the Croatian Society for Haematology of the Croatian Medical Association have made Croatian guidelines for diagnosis and treatment of myelodysplastic syndrome (MDS). MDS is a heterogeneous group of clonal hematopoietic stem cell disorders characterized by ineffective hematopoiesis, dysplasia, cytopenia and risk of transformation to acute myeloid leukemia (AML). Diagnosis is based on morphological characteristics of hematopoietic cells supplemented with the cytogenetic analysis and bone marrow flow cytometry. Due to great differences in the natural course of the disease, i.e. time to progression to AML and the expected time of survival several scoring systems have been developed to determine the disease risk. The treatment of patients with MDS is based on the risk factors of the disease as well as the individual risk of treatment

AUTOLOGOUS STEM CELL TRANSPLANTATION IN REFRACTORY OR RELAPSED DIFFUSE LARGE B CELL LYMPHOMA – A SINGLE CENTRE EXPERIENCE

Autologna transplantacija perifernih matičnih stanica zlatni je standard u liječenju refraktornog ili relapsnoga kemosenzitivnog difuznog B-velikostaničnog limfoma u pogodnih bolesnika. Cilj je ovog rada prikazati ishode autologne transplantacije perifernih matičnih stanica u bolesnika s refraktornim ili relapsnim ne-Hodgkinovim limfomom difuznoga B-velikostaničnog tipa. Retrospektivno smo analizirali podatke 62-je bolesnika liječenih autolognom transplantacijom u našem centru u razdoblju od 2000. do 2013. godine. Većina bolesnika (71%) liječena je kemoterapijom miniBEAM, a svi su primili mijeloablativnu kemoterapiju BEAM s reinfuzijom vlastitih matičnih stanica. Ukupna stopa odgovora (kom­pletna i parcijalna remisija) nakon autologne transplantacije iznosila je 75,8%. Medijan ukupnog preživljenja iznosio je 37,2 mjeseca. Medijan preživljenja bez događaja vezanih uz bolest iznosio je 16,9 mjeseci. Čimbenici statistički značajno povezani s ukupnim preživljenjem bili su aktivnost bolesti prije visokodozne spasonosne terapije, odgovor na spasonosnu terapiju. Internacionalni prognostički indeks, stadij bolesti, odgovor na autolognu transplantaciju perifernih matičnih sta­nica te radioterapija nakon autologne transplantacije. Liječenje rituksimabom nije bilo statistički značajno povezano s ishodom. U ovoj skupini bolesnika autologna je transplantacija perifernih matičnih stanica bila učinkovita u postizanju remisije i preživljenja, što je dobar i očekivan ishod navedenog postupka. Naglašavamo da je i u skupini bolesnika s kemorezistentnom bolešću autologna transplantacija bila učinkovita u 32,5% bolesnika.Autologous stem cell transplantation represents the gold standard in chemosensitive diffuse B large cell lymphoma in relapse or in refractory setting. The aim of this study was to present the outcome of peripheral autologous stem cell transplantation in patients with refractory or relapsed diffuse large B cell lymphoma. We retrospectively analysed the data of 62 patients, who underwent this procedure for the period 2000–2013. The majority of patients (71%) were treated with miniBEAM salvage chemotherapy and all received BEAM myeloablative protocol followed by the stem cell reinfusion. The overall response rate for autologous transplantation was 75.8%. Median overall survival was 37.2 months. Median event-free survival was 16.9 months. Factors associated with overall survival were state of disease prior to salvage chemotherapy, chemosensitivity of disease, International prognostic index, disease activity at the relapse, response to autologous transplantation and post-transplantation radiotherapy. The use of rituximab was not significantly correlated to the outcome. In this patient group autologous stem cell transplantation was found to be effective in achieving remission and survival showing the adequate role of this procedure in this clinical setting. We stress out that autologous stem cell transplantation was effective in 32.5% patients with chemorefractory disease after salvage therapy

GUIDELINES FOR DIAGNOSIS AND TREATMENT OF PATIENTS WITH MYELODYSPLASTIC SYNDROME

Radna skupina za mijelodisplastični sindrom Hrvatske kooperativne grupe za hematološke bolesti (KROHEM), Referalni centar Ministarstva zdravlja Republike Hrvatske za dijagnostiku i liječenje mijelodisplastičnog sindroma te Hrvatsko hematološko društvo Hrvatskoga liječničkog zbora izradili su hrvatske smjernice za dijagnozu i liječenje mijelodisplastičnog sindroma (MDS). MDS je heterogena grupa klonskih bolesti matične hematopoetske stanice karakteriziranih neučinkovitom hematopoezom, displazijom, citopenijom i rizikom od transformacije u akutnu mijeloičnu leukemiju (AML). Dijagnoza se postavlja na temelju morfoloških karakteristika stanica hematopoeze uz nadopunu citogenetskom analizom i imunofenotipizacijom koštane srži. Zbog velikih razlika u prirodnom tijeku bolesti, odnosno u vremenu do progresije u AML i u očekivanom preživljenju razvijeno je više sustava bodovanja kojima se određuje stupanj rizika od bolesti. Liječenje pacijenta s MDS-om temelji se na stupnju rizika od bolesti i individualnom riziku od liječenjaMyelodysplastic syndrome Working Group of the Croatian Cooperative Group for Hematologic Diseases (CROHEM), Referral center of the Ministry of Health of the Republic of Croatia for diagnostics and treatment of MDS, as well as the Croatian Society for Haematology of the Croatian Medical Association have made Croatian guidelines for diagnosis and treatment of myelodysplastic syndrome (MDS). MDS is a heterogeneous group of clonal hematopoietic stem cell disorders characterized by ineffective hematopoiesis, dysplasia, cytopenia and risk of transformation to acute myeloid leukemia (AML). Diagnosis is based on morphological characteristics of hematopoietic cells supplemented with the cytogenetic analysis and bone marrow fl ow cytometry. Due to great differences in the natural course of the disease, i.e. time to progression to AML and the expected time of survival several scoring systems have been developed to determine the disease risk. The treatment of patients with MDS is based on the risk factors of the disease as well as the individual risk of treatment

GUIDELINES FOR DIAGNOSIS AND TREATMENT OF PATIENTS WITH MYELODYSPLASTIC SYNDROME

Radna skupina za mijelodisplastični sindrom Hrvatske kooperativne grupe za hematološke bolesti (KROHEM), Referalni centar Ministarstva zdravlja Republike Hrvatske za dijagnostiku i liječenje mijelodisplastičnog sindroma te Hrvatsko hematološko društvo Hrvatskoga liječničkog zbora izradili su hrvatske smjernice za dijagnozu i liječenje mijelodisplastičnog sindroma (MDS). MDS je heterogena grupa klonskih bolesti matične hematopoetske stanice karakteriziranih neučinkovitom hematopoezom, displazijom, citopenijom i rizikom od transformacije u akutnu mijeloičnu leukemiju (AML). Dijagnoza se postavlja na temelju morfoloških karakteristika stanica hematopoeze uz nadopunu citogenetskom analizom i imunofenotipizacijom koštane srži. Zbog velikih razlika u prirodnom tijeku bolesti, odnosno u vremenu do progresije u AML i u očekivanom preživljenju razvijeno je više sustava bodovanja kojima se određuje stupanj rizika od bolesti. Liječenje pacijenta s MDS-om temelji se na stupnju rizika od bolesti i individualnom riziku od liječenjaMyelodysplastic syndrome Working Group of the Croatian Cooperative Group for Hematologic Diseases (CROHEM), Referral center of the Ministry of Health of the Republic of Croatia for diagnostics and treatment of MDS, as well as the Croatian Society for Haematology of the Croatian Medical Association have made Croatian guidelines for diagnosis and treatment of myelodysplastic syndrome (MDS). MDS is a heterogeneous group of clonal hematopoietic stem cell disorders characterized by ineffective hematopoiesis, dysplasia, cytopenia and risk of transformation to acute myeloid leukemia (AML). Diagnosis is based on morphological characteristics of hematopoietic cells supplemented with the cytogenetic analysis and bone marrow fl ow cytometry. Due to great differences in the natural course of the disease, i.e. time to progression to AML and the expected time of survival several scoring systems have been developed to determine the disease risk. The treatment of patients with MDS is based on the risk factors of the disease as well as the individual risk of treatment

GUIDELINES FOR DIAGNOSIS AND TREATMENT OF PATIENTS WITH MYELODYSPLASTIC SYNDROME

Radna skupina za mijelodisplastični sindrom Hrvatske kooperativne grupe za hematološke bolesti (KROHEM), Referalni centar Ministarstva zdravlja Republike Hrvatske za dijagnostiku i liječenje mijelodisplastičnog sindroma te Hrvatsko hematološko društvo Hrvatskoga liječničkog zbora izradili su hrvatske smjernice za dijagnozu i liječenje mijelodisplastičnog sindroma (MDS). MDS je heterogena grupa klonskih bolesti matične hematopoetske stanice karakteriziranih neučinkovitom hematopoezom, displazijom, citopenijom i rizikom od transformacije u akutnu mijeloičnu leukemiju (AML). Dijagnoza se postavlja na temelju morfoloških karakteristika stanica hematopoeze uz nadopunu citogenetskom analizom i imunofenotipizacijom koštane srži. Zbog velikih razlika u prirodnom tijeku bolesti, odnosno u vremenu do progresije u AML i u očekivanom preživljenju razvijeno je više sustava bodovanja kojima se određuje stupanj rizika od bolesti. Liječenje pacijenta s MDS-om temelji se na stupnju rizika od bolesti i individualnom riziku od liječenjaMyelodysplastic syndrome Working Group of the Croatian Cooperative Group for Hematologic Diseases (CROHEM), Referral center of the Ministry of Health of the Republic of Croatia for diagnostics and treatment of MDS, as well as the Croatian Society for Haematology of the Croatian Medical Association have made Croatian guidelines for diagnosis and treatment of myelodysplastic syndrome (MDS). MDS is a heterogeneous group of clonal hematopoietic stem cell disorders characterized by ineffective hematopoiesis, dysplasia, cytopenia and risk of transformation to acute myeloid leukemia (AML). Diagnosis is based on morphological characteristics of hematopoietic cells supplemented with the cytogenetic analysis and bone marrow fl ow cytometry. Due to great differences in the natural course of the disease, i.e. time to progression to AML and the expected time of survival several scoring systems have been developed to determine the disease risk. The treatment of patients with MDS is based on the risk factors of the disease as well as the individual risk of treatment

GUIDELINES FOR DIAGNOSIS AND TREATMENT OF PATIENTS WITH MYELODYSPLASTIC SYNDROME

Radna skupina za mijelodisplastični sindrom Hrvatske kooperativne grupe za hematološke bolesti (KROHEM), Referalni centar Ministarstva zdravlja Republike Hrvatske za dijagnostiku i liječenje mijelodisplastičnog sindroma te Hrvatsko hematološko društvo Hrvatskoga liječničkog zbora izradili su hrvatske smjernice za dijagnozu i liječenje mijelodisplastičnog sindroma (MDS). MDS je heterogena grupa klonskih bolesti matične hematopoetske stanice karakteriziranih neučinkovitom hematopoezom, displazijom, citopenijom i rizikom od transformacije u akutnu mijeloičnu leukemiju (AML). Dijagnoza se postavlja na temelju morfoloških karakteristika stanica hematopoeze uz nadopunu citogenetskom analizom i imunofenotipizacijom koštane srži. Zbog velikih razlika u prirodnom tijeku bolesti, odnosno u vremenu do progresije u AML i u očekivanom preživljenju razvijeno je više sustava bodovanja kojima se određuje stupanj rizika od bolesti. Liječenje pacijenta s MDS-om temelji se na stupnju rizika od bolesti i individualnom riziku od liječenjaMyelodysplastic syndrome Working Group of the Croatian Cooperative Group for Hematologic Diseases (CROHEM), Referral center of the Ministry of Health of the Republic of Croatia for diagnostics and treatment of MDS, as well as the Croatian Society for Haematology of the Croatian Medical Association have made Croatian guidelines for diagnosis and treatment of myelodysplastic syndrome (MDS). MDS is a heterogeneous group of clonal hematopoietic stem cell disorders characterized by ineffective hematopoiesis, dysplasia, cytopenia and risk of transformation to acute myeloid leukemia (AML). Diagnosis is based on morphological characteristics of hematopoietic cells supplemented with the cytogenetic analysis and bone marrow fl ow cytometry. Due to great differences in the natural course of the disease, i.e. time to progression to AML and the expected time of survival several scoring systems have been developed to determine the disease risk. The treatment of patients with MDS is based on the risk factors of the disease as well as the individual risk of treatment