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Purpose: To evaluate the influence of haemorheopheresis on anatomical and functional findings in patients with soft-drusen maculopathy. Methods: We investigated 29 eyes (16 patients) and randomized 25 eyes (16 controls) with soft-drusen maculopathy [soft, confluent and reticular drusen, drusenoid retinal pigment epithelium detachment (RPED)]. Each patient received a series of eight haemorheophereses (cascade filtration of 1.5 plasma volume) within 10 weeks. The patients were followed up using Early Treatment Diabetic Retinopathy Study (ETDRS) charts, optical coherence tomography, fluorescein angiography, electroretinography and measurements of pulsed ocular blood flow. Results: After the procedures, there was a substantial reduction in rheologically active substances [lipoproteins, α2-macroglobulin, immunoglobulin M (IgM), fibrinogen], plasma and blood viscosity. At the 1.5-year follow-up, we noticed soft drusen absorption; reattachment of drusenoid RPED and stabilization or improvement of visual acuity occurred in 72% of patients in comparison to only 39% of patients in the control group. Full-field electroretinograms showed significantly higher scotopic activity of treated patients in comparison with the control group, and mainly insignificant differences in photopic activity between both groups. Despite the significant increase of activity in the paramacular retina in treated patients, the differences in amplitudes of multifocal electroretinography (mfERG) average responses were insignificant between groups. Conclusion: Haemorheopheresis seems to be capable of changing the activity of promoters of the natural course of soft-drusen maculopathy, its development and progression. Visual acuity and electrical activity of the retina can be stabilized or even improved. The therapy has been shown to be effective and safe.

Introduction Hemaphereses are sophisticated procedures performed for many indications even in severely ill patients. Many authors consider quite necessary to register as many details as possible of treatments with therapeutic apheresis. WAA meets the requirement to compare data with centers not performing apheresis for the same diagnosis. In Czech Republic hemaphereses are used in a broad spectrum of indications. Since the year 2004 data on hemaphereses done in Czech Republic have been registered. In this paper we present a survey of our to date recordings. Patients and methods Data of performed therapeutic hemaphereses (plasma exchange, erythrocytapheresis, leukapheresis, thrombocytapheresis, photopheresis, immunopheresis, and rheopheresis) have been entered in WAA registry with many details. We have been evaluated 1289 procedures in 216 patients done in our two centers (center I, center II). Results Center I registered 129 procedures in 41 patients, center II 1260 procedures in 175 patients. The patients are divided according to centers specialization (center II has registered 12 long-term treated patients with LDL-apheresis; mean time of therapy 7.1 years and a median of 34 procedures/patient. Side-effects registered in center I and center II were 3.1% and 5.6% of the procedures, respectively. Most frequent side-effects were citrate toxicity, neurovegetative lability, problems with venous access and hypotension. All were easy to treat, no serious events or death occurred. Conclusions In Czech Republic hemaphereses are performed in a broad scale of indications and now it is nearly 5 years that data are registered in our two university centers. In 2004 we entered WAA registry because it meets the requirement to compare data with centers that do not perform aphereses for the same diagnosis. This comparison would certainly improve efficacy of the hemapheresis therapy even further. To enter WAA registry is easy, at no expense and without any problems.

The aim of this work is to arbitrate the incidence of side effects and tolerability of long lasting LDL-apheresis in familial hyperlipoproteinemia. 1200 procedures were performed and the last 463 of them were evaluated. An immunoadsorption method of LDL-apheresis was used (continuous blood cell separator Cobe Spectra; secondary device: automated adsorption-desorption ADA, Medicap; absorption columns: Lipopak). As a whole, 6.26% adverse events were found and subsequently resolved by standard symptomatic therapy. Vaso-vagal reactions (symptoms of neurovegetative lability) were the most common adverse effects, presented as malaise, weakness, slight and short-term drop in blood pressure or other general signs. They were all well controlled by symptomatic therapy. We conclude that LDL-apheresis in the hands of experienced personnel is a safe procedure. An acceptable procedure duration limit, balancing the possibility to achieve a targeted cholesterol level while still maintaining an acceptable patient tolerance, was confirmed to be 4 hours.

Uvod:LDL-afereza je metoda extrakorporalni eliminace LDL-cholesterolu, použivana u pacientů se zavažnou hyperlipidemii, ktera je rezistentni na farmakoterapii a dietni opatřeni. Metoda je narocna medicinsky i ekonomicky a je proto žadouci hledat ukazatele urcujici optimalni intenzitu i cetnost jednotlivých lecebných procedur. Z dlouhodobeho hlediska jsou ukazatele jasne (zobrazovaci metody), chybi vsak vhodne markery aktualniho stavu. Podle některých praci dochazi k normalizaci poměrů aktivovaných trombocytů. Neni vsak jasne, do jake miry jsou funkcni změny krevnich desticek vyjadřeny, jak jsou významne a jak brzy se po lecebne proceduře poměry opět horsi. Vysetřeni funkcnich změn krevnich desticek před lecebnou LDL-aferezou a po ni je potencionalně rychle a přinosne pro zprostředkovane sledovani aktivity aterogeneze a tedy přinosne k medicinsky i ekonomicky optimalnimu vedeni lecby LDL-aferezou. Urceni vhodných ukazatelů ucinnosti teto lecby na dostatecne sniženi aktivity aterosklerozy stale zůstava nedořeseným problemem. Metodika:Bylo použito vysetřeni analyzatorem Dade Behring PFA-100 (Marburg, Německo), který umožňuje měřit destickami zprostředkovanou hemostazu z nesražlive krve. Metoda napodobuje aktivaci desticek mechanickou zatěži a kolagenem in vivo. Sledujeme 9 pacientů s familiarni hypercholesterolemii (FH) (4 ženy a 5 mužů), median věku 55 let, z nich 2 homozygoti.Vysetřeni agregaci trombocytů byla provaděna standardnim způsobem na agregometru APACKS-4 (Helena Laboratories, Beaumont, USA). Předně jsme ve výzkumne sondě provedli standardni spektrum agregacni vysetřeni, kde jedine změny byly naznaceny po stimulaci ADP. Proto jsme dale vysetřili agregace s použitim 3 různých ředěni ADP - konkretně 2,5 μmol/l, 1,25 μmol/l a 0,625 μmol/l. Výsledky:Ve vsech připadech se po separaci hodnoty CT (Closure Time) prodloužily, avsak mira prodlouženi CT po procedurach nedosahla statisticke významnosti (p < 0,14). Nebyly rozdily mezi homozygoty a heterozygoty. V modifikovaných testech agregace trombocytů dochazi po afereze ke zmenseni strmosti agregacni křivky a ke sniženi maximalni amplitudy (p < 0,05). Zavěr:Procedura LDL-aferezy u nemocných s familiarni hypercholesterolemii zlepsuje poměry primarni hemostazy. Modifikovana vysetřeni agregace trombocytů by mohla být dalsim ukazatelem vhodným ke sledovani aktivity procesu u těchto těžce nemocných. Jde vsak o relativně technicky a ekonomicky narocna vysetřeni. Vysetřeni analyzatorem PFA-100 je v klinicke praxi relativně lepe dostupne, ale podle prvnich zkusenosti se zda samostatně nevhodne pro aktualni posouzeni optimalni intenzity jednotlivých procedur LDL-aferezy.