Decreased platelet membrane anisotropy in patients with adrenoleukodystrophy treated with erucic acid (22:1)-rich triglycerides

J Inherit Metab Dis. 1997 Mar;20(1):54-8. doi: 10.1023/a:1005309623452.

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.

MeSH terms

  • Adolescent
  • Adrenoleukodystrophy / blood*
  • Adrenoleukodystrophy / drug therapy
  • Adult
  • Anisotropy
  • Blood Platelets / cytology
  • Blood Platelets / metabolism*
  • Cell Membrane
  • Child
  • Erucic Acids / analysis*
  • Humans
  • Middle Aged
  • Platelet Aggregation
  • Triglycerides / therapeutic use*

Substances

  • Erucic Acids
  • Triglycerides
  • erucic acid