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Chylomicronemia syndrome (CMS) is a rare disorder characterized by the presence of chylomicrons in the fasting state causing a milky appearance of plasma, eruptive xanthomas, and hepatosplenomegaly; an acute and potentially life threatening complication is severe acute pancreatitis. The underlying defects are inborn errors of metabolism such as deficiencies of lipoprotein lipase (LPL) or apoprotein C-II (apo C-II) as well as familial hypertriglyceridemia. Moreover, CMS can be precipitated when mild hypertriglyceridemia is exacerbated by additional factors such diabetes mellitus, ethanol abuse, or pregnancy. The purpose of the present study was to retrospectively analyze the results of therapeutic plasma exchange (TPE) in 5 patients transferred to our hospital for severe acute pancreatitis due to chylomicronemia syndrome. In a total of 7 TPE sessions, on average 3,286 +/- 247 ml of plasma (i.e., about 1 patient plasma volume) were treated per session. Triglyceride (TG) levels were decreased from 4,972 +/- 2,469 mg/dl on admission to 1,614 +/- 1,276 mg/dl (-70%) after the TPE sessions, and a further decrease was achieved by conservative treatment. Part of the TG reducing effect of the treatment was probably due to heparin induced lipolysis. Acute pancreatitis was resolved in all cases, and 1 pregnant patient delivered without problems at term. In summary, 1 or 2 TPE sessions sufficed to substantially decrease the bulk of triglycerides in acutely exacerbated chylomicronemia syndrome causing a rapid resolution of acute severe pancreatitis.

The elimination of low density lipoprotein (LDL) and lipoprotein (a) (Lp[a]) by conventional LDL apheresis techniques can only be achieved in a cell-free medium and thus requires the initial separation of plasma from the blood cells. The present paper describes the first LDL hemoperfusion system which is able to adsorb LDL and Lp(a) directly from whole blood. This simplifies the procedure substantially. The adsorber consists of polyacrylate ligands linked to a modified polyacrylamide matrix. These negatively charged polyacrylate ligands interact with the positively charged apoprotein B moiety of LDL and Lp(a), which results in selective adsorption of these lipoproteins onto the column. Three hypercholesterolemic patients suffering from overt atherosclerotic complications were treated weekly by direct adsorption of lipoproteins (DALI) (n = 20 sessions each). All patients were on the highest tolerated dose of cholesterol synthesis enzyme (CSE) inhibitors. About 1.3 patient blood volumes were treated per session. The anticoagulation was performed with acid citrate dextrose (ACD-A). The following acute reductions were achieved: LDL: 66%; Lp(a): 63%; and triglycerides: 29%. High density lipoprotein (HDL) (-13%) and fibrinogen (-16%) were not substantially reduced. The sessions were essentially uneventful. Due to a low ACD-A infusion rate, no hypocalcemic episodes were registered. One patient on enalapril was treated without complications when this angiotensin converting enzyme (ACE) inhibitor was withdrawn 2 days prior to apheresis. In summary, in our hands, DALI apheresis proved to be a simple, safe, and efficient method of lipid apheresis in hypercholesterolemic patients refractory to conservative lipid lowering therapy.

Thrombotic microangiopathy (TM) is a potentially fatal complication of allogeneic bone marrow transplantation (BMT). The underlying pathophysiology is thought to be generalized endothelial cell damage caused by several factors including conditioning treatment, cyclosporin A (CsA), or graft versus host disease (GVHD). In the present retrospective study, 6 patients suffering from Grade 2 BMT-TM at a mean of 62 days post BMT were treated by 3-15 daily sessions of therapeutic plasma exchange (TPE). In most sessions, cryosupernatant (CSN) of plasma, in some fresh frozen plasma (FFP) was used as the substitution fluid. All patients suffered from acute graft versus host disease (aGVHD) of the skin, which was treated by CsA. CsA was withdrawn in all patients. TPE caused a response in 4 of 6 patients evidenced by a decrease to Grade 0 (n = 3) or 1 (n = 1) BMT-TM. Only 1 patient had mild renal insufficiency which did not improve during TPE. While all patients were dependent on platelet transfusions at baseline, the platelet counts improved in 2 of 6 patients after the TPE course. One patient did not show any response to TPE with FFP, and his disease improved only after CSN was introduced as substitution fluid (Grade 0). Four patients were still alive 175-495 days post BMT, and 2 patients died about 2-3 weeks after the end of the TPE course, 1 from cachexia and 1 from systemic aspergillosis. In summary, in this pilot study, TPE positively influenced BMT-TM, especially if CSN was used as the substitution fluid.