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

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          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 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

          Journal
          BPU
          Blood Purif
          10.1159/issn.0253-5068
          Blood Purification
          S. Karger AG
          0253-5068
          1421-9735
          2020
          July 2020
          19 March 2020
          : 49
          : 4
          : 457-467
          Affiliations
          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), fassbender@apheresis-research.org
          Article
          506506 Blood Purif 2020;49:457–467
          10.1159/000506506
          32191938
          © 2020 S. Karger AG, Basel

          Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

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

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