Relapsing and Progressive Complications of Severe Hypertriglyceridemia: Effective Long-Term Treatment with Double Filtration Plasmapheresis

Abstract

Background: Severe hypertriglyceridemia (HTG) is associated with major complications such as acute or relapsing pancreatitis (AP) and atherosclerotic cardiovascular disease (ASCVD). Rapid elimination of triglyceride (TG)-rich lipoproteins (LP) with double filtration plasmapheresis (DFPP) without need for substitution has been found to be effective for the acute, short-term treatment of HTG-induced AP. Data on the long-term use of DFPP to prevent HTG-associated complications are scarce. Objectives: To evaluate the use and efficacy of regular DFPP treatment in clinical practice for preventing recurrence of HTG-associated complications in thera py refractory patients. Methods: Retrospective multicenter study in patients with severe symptomatic drug and diet refractory HTG with regular DFPP treatment. Patients' incidence of HTG-associated pancreatic or cardiovascular complications was compared before treatment and with regular DFPP treatment. Results: Ten patients (3 female) were identified with baseline maximal TG concentrations of 2,587-28,090 mg/dL (median 5,487 mg/dL; interquartile range [IQR] 4,340-12,636). The mean observation period was 3.9 ± 3.4 years before and 3.8 ± 3.0 years after commencement of DFPP. In 5 patients, severe HTG was related to chylomicronemia, 2 patients had familial partial lipodystrophy Dunnigan, and 1 patient had additional LP(a)-hyperlipoproteinemia. The main HTG-associated complication was recurrent AP in 8 patients, including 1 patient treated during pregnancy. Two patients presented severe progressive ASCVD. With long-term DFPP treatment, the annual rate of HTGassocia ted pancreatic or cardiovascular complications declined from median 1.4 (IQR 0.7-2.6) to 0 (IQR 0.0-0.4; p < 0.005). The absolute number of events was reduced by 77%. In 6 patients (60%) episodes of AP did not occur, nor was progression of ASCVD detected clinically or by routine imag-Grupp et al.

Double filtration plasmapheresis: Review of current clinical applications

“…21 Grupp et al evaluated the efficacy of long-term regular DFPP in 10 patients for preventing recurrence of hypertriglyceridemia-associated complications. 22 The results of those studies are promising. DFPP can be applied to new diseases based on the accumulated evidence.…”

“…Chang et al applied it for 12 patients and another six patients received DFPP for prophylactic purposes 21 . Grupp et al evaluated the efficacy of long‐term regular DFPP in 10 patients for preventing recurrence of hypertriglyceridemia‐associated complications 22 . The results of those studies are promising.…”

Tandem hemodialysis and DFPP: Procedure, safety and cost‐effectiveness in patients requiring chronic hemodialysis and lipid apheresis

“…For patients with chronic hypertriglyceridemia (HTG), the goal before the DFPP session was TG <700 mg/dL. The ASFA guidelines recommend the goal of treatment to reduce time‐averaged LDL‐C by >60% from the baseline and selective LA systems are known to reduce LDL‐C levels by >60‐70% after a single session 1,12,14 . Moreover, in our department, patients treated with Tandem HD and DFPP had to undergo lab tests twice a month, before and after each DFPP session to determine the safety of hemoglobin, hematocrit, platelet, albumin (nephelometry) and fibrinogen levels and efficacy with total cholesterol, LDL‐c and TG blood levels.…”

“…When chronic treatment is indicated, a semi‐selective (DFPP) or selective TP (adsorption) method might be preferred to non‐selective SPE to avoid the need for long‐term human plasma product substitution. Bosch et al reported the first tandem experiment with HD and the heparin‐induced extracorporeal LDL‐precipitation (HELP) system 11 but none reported tandem with HD and DFPP as DFPP can be used as a long‐term treatment for LA 1,12,13 . Moreover, the tandem procedure with RRT and TP questions the blood line connection system chosen; parallel or sequential (series‐parallel) connections with considerations about maintaining dialysis efficacy which, as suggested by Kaushik et al 8 require evaluation.…”

Regional Hypertonic Citrate Anticoagulation in Membrane Therapeutic Plasma Exchange: A Case Series

“… 3 The indications for mTPE are mainly related to antibody-mediated diseases, in which the mechanical removal of antibodies mitigates briefly the disease process. 4 Other conditions that benefit from plasmapheresis are familial lipid disorders 5 , 6 and acute liver failure. 7 …”