title: Acquired lysosomal storage caused by frequent plasmapheresis procedures with hydroxyethyl starch.
abstract: BACKGROUND
  Hydroxyethyl starch (HES) solutions have largely replaced conventional plasma expanders such as human albumin and colloidal fluids. Only a few side effects have been reported and mainly concern pruritus or blood coagulation disorders. Excessive HES exposure can result in diffuse tissue storage and accumulation with foamy appearing macrophages which produce the enzyme chitotriosidase (CT). In case of massive tissue storage, this enzyme activity can reach levels comparable to those of Gaucher disease.
  
  
  METHODS
  In this single-center retrospective analysis of 11 consecutive patients receiving large amounts of HES for chronic plasmapheresis, plasma CT activity was investigated. Five patients receiving chronic intermittent plasmapheresis with conventional plasma expanders served as controls. Plasma CT activity was measured and plotted against creatinine clearance. Where available, marrow aspirate was analyzed with light microscopy to detect foamy macrophages. One patient developed a lysosomal storage disease and was examined extensively.
  
  
  RESULTS
  Conventional plasma expanders did not alter plasma CT activity. In patients with impaired renal function, frequent plasma replacement with HES resulted in an increase in plasma CT activity. In the patient with the acquired lysosomal storage disease, massive tissue infiltration with activated foamy macrophages was observed. The phagocytic capacity in this patient, however, did not seem to be altered.
  
  
  CONCLUSIONS
  Patients with impaired renal function receiving large amounts of HES exhibit an increase in plasma CT activity. Because excessive HES exposure can result in an acquired lysosomal storage disease, this should be avoided in chronic plasmapheresis procedures.
authors.affiliation: Department of Hematology, Erasmus University Medical Center, Rotterdam, The Netherlands. jja.auwerda@ikazia.nl
authors.forename: Johannes J A
authors.initials: JJ
authors.lastname: Auwerda
authors.forename: Frank W G
authors.initials: FW
authors.lastname: Leebeek
authors.forename: J H Paul
authors.initials: JH
authors.lastname: Wilson
authors.forename: Otto P
authors.initials: OP
authors.lastname: van Diggelen
authors.forename: King H
authors.initials: KH
authors.lastname: Lam
authors.forename: Pieter
authors.initials: P
authors.lastname: Sonneveld
mesh_terms: D000328:Adult; D000368:Aged; D001853:Bone Marrow / Q000201:enzymology / Q000473:pathology; D005260:Female; D005487:Foam Cells / Q000201:enzymology / Q000473:pathology; D005776:Gaucher Disease / Q000201:enzymology / Q000473:pathology; D006596:Hexosaminidases / Q000097:blood; D006801:Humans; D006895:Hydroxyethyl Starch Derivatives / Q000008:administration & dosage / Q000009:adverse effects*; D007674:Kidney Diseases / Q000097:blood / Q000201:enzymology / Q000473:pathology / Q000628:therapy*; D016464:Lysosomal Storage Diseases / Q000097:blood / Q000139:chemically induced* / Q000201:enzymology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010952:Plasma Substitutes / Q000008:administration & dosage / Q000009:adverse effects*; D010956:Plasmapheresis / Q000009:adverse effects*; D012189:Retrospective Studies
chemical_list: D006895:Hydroxyethyl Starch Derivatives; D010952:Plasma Substitutes; D006596:Hexosaminidases; C086400:chitotriosidase