title: Decreased platelet membrane anisotropy in patients with adrenoleukodystrophy treated with erucic acid (22:1)-rich triglycerides.
abstract: Low platelet count and bleeding diathesis have been observed in patients with adrenoleukodystrophy (ALD) treated with erucic acid (22:1)-rich triglycerides ("Lorenzo's oil'). To investigate possible alterations of biophysical membrane properties, we measured platelet membrane anisotropy, which is inversely related to membrane fluidity, in 16 patients with and in 3 patients without treatment. In patients on treatment, platelet membrane anisotropy was significantly decreased. Additionally, we found increased platelet concentrations of 22:1 and compromised in vitro platelet aggregation response. The decrease of platelet membrane anisotropy is probably a main cause of bleeding diathesis. Long-term haematological side-effects must be considered in ALD patients treated with Lorenzo's oil.
authors.affiliation: Department of Neuropediatrics, Children's Hospital University of Göttingen, Germany.
authors.forename: S
authors.initials: S
authors.lastname: Stöckler
authors.forename: C
authors.initials: C
authors.lastname: Opper
authors.forename: A
authors.initials: A
authors.lastname: Greinacher
authors.forename: D H
authors.initials: DH
authors.lastname: Hunneman
authors.forename: G C
authors.initials: GC
authors.lastname: Korenke
authors.forename: C J
authors.initials: CJ
authors.lastname: Unkrig
authors.forename: F
authors.initials: F
authors.lastname: Hanefeld
mesh_terms: D000293:Adolescent; D000326:Adrenoleukodystrophy / Q000097:blood* / Q000188:drug therapy; D000328:Adult; D016880:Anisotropy; D001792:Blood Platelets / Q000166:cytology / Q000378:metabolism*; D002462:Cell Membrane; D002648:Child; D004883:Erucic Acids / Q000032:analysis*; D006801:Humans; D008875:Middle Aged; D010974:Platelet Aggregation; D014280:Triglycerides / Q000627:therapeutic use*
chemical_list: D004883:Erucic Acids; D014280:Triglycerides; C049811:erucic acid