Obilježja bolesnika liječenih plazmaferezom [Characteristics of patients treated with plasmapheresis]

Frequent use of therapeutic plasmapheresis and advances in medicine and technology generated significant changes in indications, complications, method and epidemiological characteristics of patients treated with this procedure. Nevertheless many questions still remain open. Database of the National Referral Centre for therapeutic plasmapheresis and apheresis was retrospectively analyzed for changes in indications, complications, method and epidemiological characteristics of patients treated during 27 years of follow-up. An increase in number of procedures, patients and patients’ age. Neurological indications dominate, with significant changes in spectra of indications. Methods of conducting TPE also changed significantly: there is less use of unfractioned heparin, exchange volumes are larger and use of CVK more frequent. Complications occurred during 10,8 % of TPE. Use of fractionated heparin, fresh frozen plasma resulted in higher percentage of complications. That is also true for procedures done for hematological indications and in chronic program. Use of AV fistula, prophylactic replenishment of potassium and adult patients had smaller percentage of complications. Complications occurred in 7,4 % of LDL apheresis. The complexity of procedure, possible complications and high costs, ask to respect, permanently reviewe and update the guidelines for its use. National apheresis registry would contribute to improvement of the quality and safety of therapeutic apheresis and help in identifying best treatment options

PATIENT WHO DEVELOPED HEPARIN-INDUCED THROMBOCYTOPENIA TYPE II AFTER 24 YEARS ON HEMODIALYSIS

Heparinom inducirana trombocitopenija tip II (HIT) je relativno česta nuspojava terapije heparinom, a može biti uzrokom značajnog morbiditeta i mortaliteta. Heparin se rutinski primjenjuje tijekom hemodijalize, ali način primjene je drugačiji nego u općoj bolničkoj populaciji: doze su manje i ne primjenjuju se svaki dan već svaki drugi-treći dan. U literaturi ima vrlo malo podataka o prezentaciji HIT-a u odnosu na početak programa kronične hemodijalize. Većina bolesnika razvila je HIT tijekom prve godine kronične hemodijalize (prosjek 61 dan; raspon 5-390 dana). Kasnije od prosječnih 5-14 dana od početka primjene heparina, kao što je slučaj u općoj bolničkoj populaciji. Niti jedan bolesnik nije razvio HIT nakon 10 godina programa kronične hemodijalize. Prikazujemo slučaj 70-godišnje bolesnice koja je razvila HIT nakon 24 godina kronične hemodijalize i primjene heparina. HIT se prezentirao trombocitopenijom, dispnejom, bolovima u prsištu i vrućinom nakon početka hemodijalize, te trombozom femoralne vene. Nakon promjene antikoagulantne terapije u fondaparinux povisio se broj trombocita i bolesnica više nije imala tegobe tijekom hemodijalize. Prema našem saznanju, ovo je i najdulji period između početka kronične hemodijalize i pojave HIT-a do sada objavljen u literaturi. Također, ovo je prvi objavljen slučaj bolesnice na programu kronične hemodijalize koja je razvila HIT u Hrvatskoj.Heparin-induced thrombocytopenia type ii (Hit) is a clinicopathologic syndrome in which one or more clinical events are temporally related to heparin administration and caused by Hit antibodies. there are at least five different types of clinical events that are associated with Hit: thrombocytopenia; thrombosis; skin necrosis at heparin injection site; venous limb gangrene; and an acute systemic reaction that occurs 5–30 min after intravenous bolus of heparin. Hit typically presents 5–14 days after initiation of heparin therapy, later onset is unusual. Heparin is a routine anticoagulant in hemodialysis but administration is different than in surgical and other medical population. doses are lower and administered every other day, yet hemodialysis patients receive heparin for years. relationship between dialysis vintage and Hit-antibody positivity has been analyzed in two studies. in national survey of Hit in hemodialysis population of the united kingdom mean time between starting hemodialysis and development of Hit was 61 days (5–390 days). japanese authors also found greatest incidence of Hit antibody positivity in patients who were on hemodialysis for less than 1 year, none of patients on hemodialysis for more than 10 years was Hit-antibody positive. We present a case of 70-years old female who developed Hit after 24 years of hemodialysis and exposure to heparin. first 22 years she was receiving unfractionated heparin for anticoagulation during hemodialysis sessions. afterwards her therapy was changed to low molecular weight heparin. Last 12 years she has tunneled cuffed catheter which was also filled with unfractionated heparin. she had a history of severe renal osteodistrophy and severe aortal valve stenosis, hypothyreosis, thrombosis of both subclavian veins and partial colon resection due to mesenterial artery thrombosis. Her thrombocyte count was low, but despite extensive work-up which included Hit antibody detection, no cause could be identified. she started complaining of flushing, dyspnea and chest pain that developed several minutes after start of hemodialysis and stopped spontaneously during or after hemodialysis. symptoms were attributed to her heart disease and she was hospitalized for cardiac reevaluation. thrombosis of right superficial and commune femoral vein was diagnosed as well as further worsening of thrombocytopenia. Hit antibodies were assessed again and they were positive. anticoagulation during hemodialysis was changed to fondaparinux and catheter filling to citrate. afterwards symptoms during hemodialysis disappeared and thrombocyte count recovered. Hit type ii is a rare but potentially fatal syndrome that can develop years after start of heparin therapy. to our knowledge, this is the patient with longest hemodialysis vintage and newly diagnosed Hit. this is also the first case of patient on hemodialysis that developed Hit in croatia published to date

NEFROPATIJA UZROKOVANA BK VIRUSOM U BOLESNIKA S TRANSPLANTIRANIM SRCEM: PRVI DOKUMENTIRANI SLUČAJ U HRVATSKOJ

As outcomes following heart transplantation have improved significantly over the last years, chronic kidney disease has become an increasingly prevalent complication in this population. Polyomavirus-associated nephropathy (PVAN) of native kidneys has also been recognized increasingly as a cause of kidney failure. We report the first case of PVAN occurring in the native kidneys of a solid-organ transplant recipient in Croatia as the eighth case described in the literature worldwide. A 65-year-old female with dilatative cardiomyopathy and good kidney function had a heart transplanted in 2012. Initial immunosuppressive therapy consisted of antithymocyte immunoglobulin with cyclosporine, mycophenolate mofetil and corticosteroids. Soon after transplantation, her kidney function began to fail progressively. Biopsy of the native kidneys revealed PVAN, and everolimus was introduced in immunosuppressive therapy. Nevertheless, her renal dysfunction progressed and she is now being evaluated for cadaveric kidney transplantation. PVAN should be considered in the differential diagnosis of new-onset renal failure following non-kidney solid organ transplantation. Early diagnosis is essential for prevention of irreversible renal damage.Unazad nekoliko godina sa značajnim poboljšanjem preživljenja bolesnika s transplantiranim srcem kronično zatajenje bubrega postalo je sve češća komplikacija u toj populaciji. Nefropatija uzrokovana poliomavirusom (PVAN) nativnih bubrega također se sve češće prepoznaje kao uzrok zatajenja bubrega. Prikazujemo prvi slučaj PVAN nativnih bubrega kod primatelja transplantata solidnog organa u Hrvatskoj i osmi takav slučaj dosad opisan u literaturi. Bolesnici u dobi od 65 godina s dilatativnom kardiomiopatijom i dobrom bubrežnom funkcijom je 2012. godine transplantirano srce. Inicijalna imunosupresivna terapija sastojala se od antitimocitnog imunoglobulina s ciklosporinom, mikofenolat mofetilom i kortikosteroidima. Ubrzo nakon transplantacije dolazi do zatajenja bubrega. Biopsijom nativnih bubrega postavljena je dijagnoza PVAN, a u imunosupresivnu terapiju je uveden everolimus. Usprkos tome dolazi do daljnjeg napredovanja zatajenja bubrega i bolesnica je trenutno u pripremi za kadaveričnu transplantaciju bubrega. PVAN treba razmotriti u diferencijalnoj dijagnozi novonastalog zatajenja bubrega nakon transplantacije solidnih organa. Rana dijagnoza PVAN je bitna u sprječavanju razvoja ireverzibilnog bubrežnog zatajenja

TEMPORARY AND PERMANENT CENTRAL VENOUS CATHETERS FOR HEMODIALYSIS

Iako se arteriovenska fistula smatra optimalnim pristupom krvožilju, uporaba trajnih i privremenih dijaliznih katetera za hemodijalizu je u stalnom porastu. Razlog tomu je sve starija populacija kod koje se započinje postupak trajnog bubrežnog nadomjesnog liječenja i kao posljedica toga starija i neprikladna vaskulatura za izradu arterio-venskih fistula. Komplikacije su moguće prilikom postavljanja katetera, ali i kod postavljenih katetera što prije svega uključuje tromboze i infekcije. Na ozbiljnost komplikacija utječe iskustvo osobe koja postavlja kateter, ali i kvaliteta te lokalizacija krvne žile na kojoj se zahvat izvodi. U ovom preglednom članku opisano je praćenje kvalitete rada dijaliznih katetera, mjesto izbora postavljanja te metode rješavanja tromboziranih katetera i lokalnih i sistemnih infekcija. Preporučuju se svakodnevna procjena funkcije, pravilna njega i higijena dijaliznih katetera.The usage of temporary and permanent dialysis catheters for hemodialysis vascular access has been on continual increase. The reason for this increase is aging population on hemodialysis with blood vessels inappropriate for arteriovenous fistula creation. Complications may occur during catheter insertion as well as in the already inserted catheters, e.g., thrombosis and infections. The severity of complications is determined by experience of the operator as well as the quality and localization of blood vessels. Monitoring of dialysis catheter function, choice of the site of catheter insertion and methods of salvaging catheters from thrombosis and treating catheter-related local and systemic infections are described in this paper. Constant evaluation, proper care and hygiene of dialysis catheters are highly recommended

Characteristics of patients treated with plasmapheresis

Učestalija primjena terapijske plazmafereze te napredak medicine i tehnologije donijeli su značajne promjene metode, indikacija, komplikacija i obilježja bolesnika liječenih ovim postupkom. Unatoč tome, mnoga pitanja i dalje su ostala otvorena. Retrospektivno je analizirana baza podataka terapijskih afereza Referentnog centra za Ministarstva zdravstva za nadomještanje bubrežne funkcije, plazmaferezu i aferezu Republike Hrvatske nastala tijekom 27 godina praćenja. Istražena je dinamika promjena u indikacijama, komplikacijama, metodi i epidemiološkim osobitostima bolesnika podvrgnutih postupcima. Zabilježeno je povećanje broja postupaka i bolesnika te njihove dobi. Dominiraju neurološke indikacije, uz značajne promjene u spektru indikacija. Značajno su se promijenile i metode provođenja TPF-a: manja se rabi nefrakcionirani heparin, volumeni izmjene sve su veći, a postupci se češće provode putem CVK. Komplikacije su zabilježene u 10,8 % postupaka. Veći udio komplikacija zabilježen je tijekom postupaka u kojima se rabi niskomolekularni heparin i SSP te postupaka koji se provode zbog hematoloških indikacija i u kroničnom programu. Manji udio zabilježen je tijekom postupaka putem AV fistule, uz profilaktičku nadoknadu kalija i kod bolesnika srednje dobi. Tijekom LDL afereza komplikacije su zabilježene u 7,4 % postupaka. Složenost postupaka afereze, moguće komplikacije, ali i visoka cijena zahtijevaju da se poštuju, trajno preispituju i dorađuju smjernice za njezinu uporabu. Nacionalni registar afereza Republike Hrvatske pridonio bi unapređenju kvalitete i sigurnosti provođenja terapijske plazmafereze, te pomogao u pronalaženju najbolje terapijske mogućnosti.Frequent use of therapeutic plasmapheresis and advances in medicine and technology generated significant changes in indications, complications, method and epidemiological characteristics of patients treated with this procedure. Nevertheless many questions still remain open. Database of the National Referral Centre for therapeutic plasmapheresis and apheresis was retrospectively analyzed for changes in indications, complications, method and epidemiological characteristics of patients treated during 27 years of follow-up. An increase in number of procedures, patients and patients’ age. Neurological indications dominate, with significant changes in spectra of indications. Methods of conducting TPE also changed significantly: there is less use of unfractioned heparin, exchange volumes are larger and use of CVK more frequent. Complications occurred during 10,8 % of TPE. Use of fractionated heparin, fresh frozen plasma resulted in higher percentage of complications. That is also true for procedures done for hematological indications and in chronic program. Use of AV fistula, prophylactic replenishment of potassium and adult patients had smaller percentage of complications. Complications occurred in 7,4 % of LDL apheresis. The complexity of procedure, possible complications and high costs, ask to respect, permanently reviewe and update the guidelines for its use. National apheresis registry would contribute to improvement of the quality and safety of therapeutic apheresis and help in identifying best treatment options

Characteristics of patients treated with plasmapheresis

Učestalija primjena terapijske plazmafereze te napredak medicine i tehnologije donijeli su značajne promjene metode, indikacija, komplikacija i obilježja bolesnika liječenih ovim postupkom. Unatoč tome, mnoga pitanja i dalje su ostala otvorena. Retrospektivno je analizirana baza podataka terapijskih afereza Referentnog centra za Ministarstva zdravstva za nadomještanje bubrežne funkcije, plazmaferezu i aferezu Republike Hrvatske nastala tijekom 27 godina praćenja. Istražena je dinamika promjena u indikacijama, komplikacijama, metodi i epidemiološkim osobitostima bolesnika podvrgnutih postupcima. Zabilježeno je povećanje broja postupaka i bolesnika te njihove dobi. Dominiraju neurološke indikacije, uz značajne promjene u spektru indikacija. Značajno su se promijenile i metode provođenja TPF-a: manja se rabi nefrakcionirani heparin, volumeni izmjene sve su veći, a postupci se češće provode putem CVK. Komplikacije su zabilježene u 10,8 % postupaka. Veći udio komplikacija zabilježen je tijekom postupaka u kojima se rabi niskomolekularni heparin i SSP te postupaka koji se provode zbog hematoloških indikacija i u kroničnom programu. Manji udio zabilježen je tijekom postupaka putem AV fistule, uz profilaktičku nadoknadu kalija i kod bolesnika srednje dobi. Tijekom LDL afereza komplikacije su zabilježene u 7,4 % postupaka. Složenost postupaka afereze, moguće komplikacije, ali i visoka cijena zahtijevaju da se poštuju, trajno preispituju i dorađuju smjernice za njezinu uporabu. Nacionalni registar afereza Republike Hrvatske pridonio bi unapređenju kvalitete i sigurnosti provođenja terapijske plazmafereze, te pomogao u pronalaženju najbolje terapijske mogućnosti.Frequent use of therapeutic plasmapheresis and advances in medicine and technology generated significant changes in indications, complications, method and epidemiological characteristics of patients treated with this procedure. Nevertheless many questions still remain open. Database of the National Referral Centre for therapeutic plasmapheresis and apheresis was retrospectively analyzed for changes in indications, complications, method and epidemiological characteristics of patients treated during 27 years of follow-up. An increase in number of procedures, patients and patients’ age. Neurological indications dominate, with significant changes in spectra of indications. Methods of conducting TPE also changed significantly: there is less use of unfractioned heparin, exchange volumes are larger and use of CVK more frequent. Complications occurred during 10,8 % of TPE. Use of fractionated heparin, fresh frozen plasma resulted in higher percentage of complications. That is also true for procedures done for hematological indications and in chronic program. Use of AV fistula, prophylactic replenishment of potassium and adult patients had smaller percentage of complications. Complications occurred in 7,4 % of LDL apheresis. The complexity of procedure, possible complications and high costs, ask to respect, permanently reviewe and update the guidelines for its use. National apheresis registry would contribute to improvement of the quality and safety of therapeutic apheresis and help in identifying best treatment options

Characteristics of patients treated with plasmapheresis

Učestalija primjena terapijske plazmafereze te napredak medicine i tehnologije donijeli su značajne promjene metode, indikacija, komplikacija i obilježja bolesnika liječenih ovim postupkom. Unatoč tome, mnoga pitanja i dalje su ostala otvorena. Retrospektivno je analizirana baza podataka terapijskih afereza Referentnog centra za Ministarstva zdravstva za nadomještanje bubrežne funkcije, plazmaferezu i aferezu Republike Hrvatske nastala tijekom 27 godina praćenja. Istražena je dinamika promjena u indikacijama, komplikacijama, metodi i epidemiološkim osobitostima bolesnika podvrgnutih postupcima. Zabilježeno je povećanje broja postupaka i bolesnika te njihove dobi. Dominiraju neurološke indikacije, uz značajne promjene u spektru indikacija. Značajno su se promijenile i metode provođenja TPF-a: manja se rabi nefrakcionirani heparin, volumeni izmjene sve su veći, a postupci se češće provode putem CVK. Komplikacije su zabilježene u 10,8 % postupaka. Veći udio komplikacija zabilježen je tijekom postupaka u kojima se rabi niskomolekularni heparin i SSP te postupaka koji se provode zbog hematoloških indikacija i u kroničnom programu. Manji udio zabilježen je tijekom postupaka putem AV fistule, uz profilaktičku nadoknadu kalija i kod bolesnika srednje dobi. Tijekom LDL afereza komplikacije su zabilježene u 7,4 % postupaka. Složenost postupaka afereze, moguće komplikacije, ali i visoka cijena zahtijevaju da se poštuju, trajno preispituju i dorađuju smjernice za njezinu uporabu. Nacionalni registar afereza Republike Hrvatske pridonio bi unapređenju kvalitete i sigurnosti provođenja terapijske plazmafereze, te pomogao u pronalaženju najbolje terapijske mogućnosti.Frequent use of therapeutic plasmapheresis and advances in medicine and technology generated significant changes in indications, complications, method and epidemiological characteristics of patients treated with this procedure. Nevertheless many questions still remain open. Database of the National Referral Centre for therapeutic plasmapheresis and apheresis was retrospectively analyzed for changes in indications, complications, method and epidemiological characteristics of patients treated during 27 years of follow-up. An increase in number of procedures, patients and patients’ age. Neurological indications dominate, with significant changes in spectra of indications. Methods of conducting TPE also changed significantly: there is less use of unfractioned heparin, exchange volumes are larger and use of CVK more frequent. Complications occurred during 10,8 % of TPE. Use of fractionated heparin, fresh frozen plasma resulted in higher percentage of complications. That is also true for procedures done for hematological indications and in chronic program. Use of AV fistula, prophylactic replenishment of potassium and adult patients had smaller percentage of complications. Complications occurred in 7,4 % of LDL apheresis. The complexity of procedure, possible complications and high costs, ask to respect, permanently reviewe and update the guidelines for its use. National apheresis registry would contribute to improvement of the quality and safety of therapeutic apheresis and help in identifying best treatment options

PREPARATION OF HIGHLY HLA-SENSITIZED PATIENT FOR HEART TRANSPLANTATION: FIRST DOCUMENTED CASE IN CROATIA

Prisutnost antitijela na ljudske leukocitne antigene (Human Leukocyte Antigens – HLA) je značajan terapijski izazov u transplantaciji organa. Senzibiliziranost (Panel Reactive Antibody – PRA >10%) primatelja transplantiranog srca povezana je s nastankom niza kliničkih sindroma koji utječu na kratkotrajno, a moguće i na dugotrajno preživljenje bolesnika. Prikazujemo slučaj modulacije imunog sustava u pripremi za transplantaciju srca visoko senzibilizirane bolesnice. Prema našem saznanju, to je prvi slučaj provedene terapije desenzibilizacije u transplantaciji solidnih organa na području Republike Hrvatske. Bolesnici je zbog dekompenzirane primarne dilatativne kardiomiopatije ugrađen sustav za potporu lijeve klijetke kako bi preživjela do transplantacije srca. Obradom za listu za transplantaciju dijagnosticirana je gljivična pneumonija i visoki stupanj senzibilizacije na HLA (PRA 97%). Protokol desenzibilizacije uključivao je mikofenolatmofetil, takrolimus, terapijske plazmafereze i imunoglobuline uz ranije uključenu antifungalnu terapiju. Navedenim smo postupkom smanjili PRA na svega 6%. Nažalost, tijekom sljedećih nekoliko tjedana nije nađen odgovarajući davatelj, te je bolesnica umrla zbog multiorganskog zatajenja. Izbjegavanje nastanka alosenzibilizacije ranom transplantacijom, izbjegavanjem transfuzija, upotrebom koncentrata eritrocita sa smanjenim brojem leukocita izuzetno je važno kod potencijalnih primatelja transplantiranih organa kako bi se smanjilo vrijeme čekanja na adekvatan organ, mogućnost razvoja brojnih komplikacija i smanjilo smrtnost bolesnika.The presence of human leukocyte antigen (HLA) poses a significant therapeutic challenge in solid organ transplantation. Sensitized patients (panel reactive antibody, PRA >10%) receiving a heart transplant are at an increased risk of developing several clinical syndromes that influence short-term, and possibly also long-term patient survival. We present a case of immune system modulation in a highly sensitized patient awaiting heart transplantation. To our knowledge, this is the first case of desensitization therapy in solid organ transplantation done in Croatia. The patient with decompensated primary dilated cardiomyopathy received left ventricular assist device as a bridge to heart transplantation. Pre-transplantation evaluation revealed the presence of fungal pneumonia and high sensitization to HLA antibodies (PRA 97%). Desensitization protocol consisted of mycophenolate mofetil, tacrolimus, intravenous immune globulin and preoperative plasmapheresis along with previously initiated antifungal therapy. We withheld the application of rituximab until after resolution of pneumonia, but our protocol lowered PRA to 6% even without it. Unfortunately, during the next several weeks, a suitable donor was not found and our patient died form multiorgan failure. Avoidance of allosensitization by early referral to transplantation center, early transplantation, and avoidance of transfusions are very important when treating a potential transplant recipient. In this way, waiting time to ransplantation, development of posttransplantation complications and mortality are reduced