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

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          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 thera­py 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 HTG-associa­ted 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 imaging techniques. DFPP was effective in the elimination of TG-rich LP from plasma, and was safe and well-tolerated. Conclusion: Long-term, regular DFPP treatment resulted in stabilization of patients with severe HTG and related recurrent AP or progression of ASCVD, who were refractory to conventional dietary and drug therapy.

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          Author and article information

          Blood Purif
          Blood Purification
          S. Karger AG
          July 2020
          19 March 2020
          : 49
          : 4
          : 457-467
          aDepartment of Nephrology, Academic Teaching Hospital Bamberg, Bamberg, Germany
          bDepartment of Nephrology and Rheumatology, Georg-August University Goettingen, Göttingen, Germany
          cDepartment of Nephrology, St. Marien Hospital Vechta GmbH, Vechta, Germany
          dDepartment of Internal Medicine II, Hospital of Barmherzige Brüder, Trier, Germany
          eDepartment of Internal Medicine IV-Nephrology, Saarland University Hospital, Homburg, Germany
          fJoint Practice of Internal Medicine, Witten, Germany
          gKiel Kidney and Vascular Center, Kiel, Germany
          hNephrological Center, Villingen-Schwennigen, Germany
          iDialysis and Lipid Center North Rhine, Mülheim an der Ruhr, Germany
          jDialysis and Lipid Center North Rhine, Essen, Germany
          kApheresis Research Institute, Cologne, Germany
          lFirst Department of Internal Medicine, University of Mainz, Mainz, Germany
          mDepartment of Nephrology, University Hospital Essen, University of Duisburg-Essen, Essen, Germany
          Author notes
          *Dr. Cordula M. Fassbender, Apheresis Research Institute, Stadtwaldgürtel 77, DE–50935 Cologne (Germany),
          506506 Blood Purif 2020;49:457–467
          © 2020 S. Karger AG, Basel

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          Page count
          Figures: 3, Tables: 1, Pages: 11
          Research Article


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