M. Lanska

Beneficial effect of plasma exchange in the treatment of toxic epidermal necrolysis: A series of four cases

Introduction: Toxic epidermal necrolysis (TEN) is a rare, life‐threatening disease with a high mortality rate that is linked to drug toxicity. There is a lack of data about the underlying pathophysiologic mechanisms and treatment options. The only widely accepted treatment of TEN is withdrawal of the offending drug followed by supportive care. The potential roles of corticosteroids, intravenous immunoglobulin (IVIG) and plasmapheresis (TPE) remain controversial. Aims: We present four patients with severe TEN (all with >80% involvement of body surface) who were treated with TPE following unsuccessful treatment with corticosteroids/IVIG. Methods: TPE was performed using a COBE Spectra blood cell separator. ACD‐A was used as anticoagulant fluid and the target‐washed plasma volume was one body volume. Plasma was replaced by a 5% solution of human albumin + Ringers lactate. Results: The mean number of TPE sessions was 5.25 ± 2.22 (range 3–8). Drugs were implicated as an etiologic agent in each case. TPE led to prompt improvement of acute condition and general health as well as halting of disease progression. Additionally, the restoration of the epithelium began in all four patients. Conclusion: Plasmapheresis should be considered as an alternative treatment modality for patients with the most severe form of TEN if initial treatment with other agents, including corticosteroids and/or IVIG, fails. Drugs were suspected to be the cause of TEN in all four cases. J. Clin. Apheresis, 2012.

Rheohaemapheresis in the treatment of nonvascular age-related macular degeneration

PURPOSE To evaluate the experience with rheohaemapheresis (RH) in the treatment of age-related macular degeneration (AMD). METHODS Thirty-eight patients were each treated with 8 procedures of RH (14 males, 24 females). The control group consisted of 34 random patients (30 females, 4 males) with the dry form of AMD but not treated by RH. Our modification of the cascade method (named rheohaemapheresis) was used for plasma separation. After plasma separation (blood cell separator, Cobe Spectra, Denver, CO, USA), the separated plasma was pumped through a rheofilter (Evaflux 4A, Kuraray, Osaka, Japan) to remove lipoproteins and other high-molecular-weight rheologic factors. RESULTS In treated patients, best-corrected visual acuity (BCVA) increased significantly from 0.61 (0.06-1.00) to 0.68 (0.35-1.00) after 2.5 years (p = 0.035). We found no significant changes or differences in scotopic activity, whereas cone response and paramacular activity in the more peripheral region between 14° and 22° of eccentricity were significantly higher in treated patients after 2.5 years. CONCLUSION RH therapy favourably influenced BCVA. During 2.5 years after the therapy, no progression of dry to wet AMD was observed in our patients. RH reduced the area of drusenoid retinal pigment epithelium detachment (which increased during the natural course of dry form AMD). RH influenced rheological markers and probably improved metabolism in the affected retinal areas which lead to the aforementioned positive results.

Pregnancy in homozygous familial hypercholesterolemia – Importance of LDL-apheresis

INTRODUCTION Rare cases of pregnancy in women with homozygous familial hypercholesterolemia (HFH) have been reported. HFH might pose significant risks for the mother and her fetus. Statins, the most potent agents for low-density lipoprotein (LDL) cholesterol reduction, are contraindicated; thus lipoprotein apheresis remains the only effective treatment. CASE REPORT We report on a 34-year-old pregnant woman with HFH who was treated throughout the entire pregnancy by lipoprotein apheresis (immunoadsorption method). Increasing levels of LDL-cholesterol were stabilized at 9-10 mmol/L by lipoprotein apheresis (performed every 10 days). No complications were observed during the treatment procedures. Monitoring of the fetus revealed no impairment of the umbilical cord and blood flow in the uterine arteries, as well as no intrauterine growth retardation. The delivery was spontaneous and the child was breastfed for two months. CONCLUSION Intensive treatment by lipoprotein apheresis is an effective and safe therapeutic strategy during pregnancy, even in severe cases of HFH, as it can stabilize progressively increasing lipoprotein levels and prevent severe complications.

The decrease of mean platelet volume after extracorporeal LDL-cholesterol elimination

OBJECTIVE Mean platelet volume is arousing increasing interest as a new independent cardiovascular risk factor. Large platelets are likely to be more reactive. If mean platelet volume would drop after LDL-lowering therapy, decreased MPV could be one of the markers of successful therapy. Therefore, we investigated mean platelet volume after extracorporeal LDL-cholesterol elimination. METHODS Mean platelet volume was investigated in patients with severe familial hypercholesterolemia long-term treated (3-12 years) by LDL-apheresis (immunoapheresis) or cascade filtration. Plasma was obtained by centrifugation. Adsorbers Lipopak 400 were used for immunoapheresis and filters Evaflux 4A were used for cascade filtration. 95 pair samples were measured (before and after the procedures) in a group of 12 patients--each patient 8 times in 4 years. RESULTS Mean platelet volume before the procedures was 10.891 fl, CI 10.25-11.53. Mean platelet volume after the procedures decreased--10.478 fl, CI 09.84-11.11. The difference is statistically significant (p = 0.036). Mean platelet volume did not correlate with age, sex, platelet count, duration of therapy. At the same time, we used rheohemapheresis in the therapy of 40 patients with age-related macular degeneration. But mean platelet volume was not changed. CONCLUSION Mean platelet volume is easily available and is often disregarded, and sometimes may suggest the need for a careful assessment in patients with familial hypercholesterolemia. Mean platelet volume could be one of the markers of therapeutic efficacy in patients with familial hypercholesterolemia treated by extracorporeal LDL-cholesterol elimination that is simple and inexpensive.

Changes of the complement system and rheological indicators after therapy with rheohemapheresis

INTRODUCTION In the last 10 years, many studies have been published on the role of the complement system in microcirculation disorders. However, as for the changes of complement components after rheohemapheresis, there is still a lack of detailed data in the literature. Complement changes may play an important role in pathogenesis of some microcirculation disorders, such as age-related macular degeneration and acute hearing loss. The objective of this study was to investigate the effect of rheohemapheresis on the basic complement pathways. PATIENTS AND METHODS 32 patients were treated with rheohemapheresis, including 16 patients (10 men and 6 women) for age-related macular degeneration (AMD), mean age 69.7 ± 6.06 years (range 62-87 years) and 16 patients (11 men and 5 women) aged 56.4 ± 11.5 (range 34-73 years) for acute hearing loss. RESULTS Rheohemapheresis led to a significant drop of all three complement-activation pathways in both groups of patients. Moreover, complement factor H was also reduced. CONCLUSION The observed reduction in all three basic complement activation pathways after rheohemapheresis could be clinically important. The search continues both to find substances which influence complement systems and to develop more effective new drugs that require less frequent administration and that provide improved intraocular therapy for AMD patients.

Timing of Peripheral Blood Stem Cell Yield: Comparison of Alternative Methods with the Classic Method for CD34 + Cell Determination

Hematopoietic stem cells (HSCs), still represent a certain mystery in biology, have a unique property of dividing into equal cells and repopulating the hematopoietic tissue. This potential enables their use in transplantation treatments. The quality of the HSC grafts for transplantation is evaluated by flow cytometric determination of the CD34+ cells, which enables optimal timing of the first apheresis and the acquisition of maximal yield of the peripheral blood stem cells (PBSCs). To identify a more efficient method for evaluating CD34+ cells, we compared the following alternative methods with the reference method: hematopoietic progenitor cells (HPC) enumeration (using the Sysmex XE-2100 analyser), detection of CD133+ cells, and quantification of aldehyde dehydrogenase activity in the PBSCs. 266 aphereses (84 patients) were evaluated. In the preapheretic blood, the new methods produced data that were in agreement with the reference method. The ROC curves have shown that for the first-day apheresis target, the optimal predictive cut-off value was 0.032 cells/mL for the HPC method (sensitivity 73.4%, specificity 69.3%). HPC method exhibited a definite practical superiority as compared to other methods tested. HPC enumeration could serve as a supplementary method for the optimal timing of the first apheresis; it is simple, rapid, and cheap.

Long-Term Outcomes of Rheohaemapheresis in the Treatment of Dry Form of Age-Related Macular Degeneration

Purpose. Determining long-term effects of rheohaemapheresis on the dry form of age-related macular degeneration. Methods. This study evaluates 19 patients, average age of 67.6 years, treated with rheohaemapheresis and 18 patients, average age of 72.8 years, comprising the control group. Minimum follow up period was 3.5 years. Each treated patient received a series of 8 sessions of rheohaemapheresis of 1.5 plasma volumes within 10 weeks. We measured the drusenoid pigment epithelium detachment (DPED), best-corrected visual acuity (BCVA), electroretinography (ERG), and rheological parameters. Results. In the treatment group, the baseline BCVA was 0.74 (0.36–1.0) 95% CI and BCVA after 3.5 years was 0.79 (0.41–1.0) 95% CI (P = 0.726). In the control group, the baseline BCVA was 0.71 (0.15–1.0) 95% CI and BCVA after 3.5 years decreased to 0.7 (0.32–0.87) 95% CI (P = 0.031). Baseline DPED was 6.78 ± 3.79 mm2; after 3.5 years, it decreased to 4.13 ± 3.84 mm2 (P < 0.001). In the control group, the baseline DPED was 4.09 ± 3.48 mm2; after 3.5 years, it increased to 6.69 ± 4.2 mm2 (P = 0.001). We noted increasing levels of positive wave peaking at 50 milliseconds (P50) after treatment (P = 0.022) and a stable amplitude of photopic responses of treated patients. Conclusion. Over the long term, rheohaemapheresis reduced the DPED, improved the function of photoreceptors, and prevented the decline of BCVA.

The importance of rheological parameters in the therapy of the dry form of age-related macular degeneration with rheohaemapheresis

To date, rheological treatment is the only chance to control the advanced dry form of age-related macular degeneration and arrest its progression to legal blindness. Rheohaemapheresis can change the main rheological parameters, blood and plasma viscosity, as well as change erythrocyte aggregability, improve erythrocyte flexibility and lead to substantial improvement when other methods of therapy fail. In this study, we describe changes in the levels of rheological efficacy indicators after rheohaemapheresis and their clinical significance in the dry form of age-related macular degeneration (AMD). Seventy-two patients with AMD were randomised; 34 controls, and 38 patients were treated with rheohaemapheresis (separator Cobe Spectra + Evaflux filter). After the procedures, α2-macroglobulin levels decreased by approximately 58%, fibrinogen by approximately 65%, IgM by approximately 67%, LDL cholesterol by approximately 71%, apolipoprotein B by approximately 65%, and lipoprotein (a) by approximately 42%. These decreases correspond with a decrease in blood and plasma viscosity (14/12%), clinical improvement (arrest of disease progression, even visual improvement in some cases), and heretofore-unreported improvement (even reattachment) of drusen retinal pigment epithelium detachment. Our modification of rheohaemapheresis is safe (5.4% of patients experienced clinically insignificant side effects).

Extracorporeal immunoglobulin elimination for the treatment of severe myasthenia gravis.

Myasthenia gravis (MG) is a neuromuscular disorder leading to fluctuating muscle weakness and fatigue. Rarely, long-term stabilization is not possible through the use of thymectomy or any known drug therapy. We present our experience with extracorporeal immunoglobulin (Ig) elimination by immunoadsorption (adsorbers with human Ig antibodies). Acetylcholine receptor antibodies (AChRAs) were measured during long-term monitoring (4.7 ± 2.9 years; range 1.1–8.0). A total of 474 samples (232 pairs) were analyzed, and a drop in AChRA levels was observed (P = .025). The clinical status of patients improved and stabilized. Roughly 6.8% of patients experienced clinically irrelevant side effects. The method of Ig elimination by extracorporeal immunoadsorption (IA) is a clinical application of the recent biotechnological advances. It offers an effective and safe therapy for severe MG even when the disease is resistant to standard therapy.

Does mean platelet volume really increase in sudden sensorial hearing loss

Dear Editor, Ulu et al. [1] recently published an interesting paper in this journal on the subject of mean platelet volume in patients with sudden idiopathic sensorial hearing loss (SISHL). The authors found a significant increase in the volume of circulating platelets in 40 patients treated in the past 6 years when compared to a group of 40 healthy individuals of identical age and gender. It has been proposed that increased mean platelet volume (and increased platelet activity) may be linked with sudden idiopathic sensorineural hearing loss and that it may indicate ischemia or atherosclerosis as possible causes of SISHL. The publication of Ulu et al. [1] raised considerable interest among readers: editors repeatedly received Letters to the Editor with consensual as well as contradictory opinions, and Ulu et al. published additional interesting ideas in their responses to these Letters to the Editor. Pirodda et al. [2] sent a Letter to the Editor of the European Archives of Oto-Rhino-Laryngology with interesting thoughts on serious functional disorders that can also, in addition to organic disorders, lead to SISHL. It is necessary to respect the relationships between circulation and homeostasis in the inner ear and respect its anatomical structures, the terminal vasculature of the inner ear, and the high-energy demands of this sensory organ. The vascular risk-free population has been extensively studied over the past years and the possibility of cochlear damage associated with SISHL in young and healthy subjects with low blood pressure values was demonstrated. In such cases, abrupt lowering of blood pressure followed by abnormal vasoconstriction could be responsible for the damage [3, 4]. It outlines a link between transiently insufficient peripheral perfusion of systemic origin and acute inner ear sufferance—both detected, for example, in subjects submitted to aggressive anti-hypertensive therapy [5]. Balta et al. [6] sent another Letter to the Editor and pointed to a clinically significant—the question of practical clinical use of mean platelet volume is not simple and comprehensive; the full extent of any assessment must be carried out in relation to a number of other factors. It is of course advantageous that mean platelet volume testing is available during common complete blood count examinations. It is a routine, simple and inexpensive technique that gives a lot of information about the patient’s formed blood contents: the red and white cells, platelets, the counts and dimensions of subgroups of cells as well as parameters, including distribution widths, mean platelet volume, etc. That is why MPV is one of the most widely used laboratory markers related to platelet function based on the inflammatory conditions. As Balta et al. [6] reported, it is advantageous that mean platelet volume is such a simple and accessible marker in addition to being of key importance in the pathophysiology of a number of complex and serious conditions, such as coronary heart disease, endothelial dysfunction and others. Platelet parameters can be affected by coronary risk factors, including age, obesity, This comment refers to the article available at doi:10.1007/s00405013-2348-9.