DECREASED PLATELET MEMBRANE ANISOTROPY IN PATIENTS WITH ADRENOLEUKODYSTROPHY TREATED WITH ERUCIC-ACID (22 1)-RICH TRIGLYCERIDES/

Low platelet count and bleeding diathesis have been observed in patien ts with adrenoleukodystrophy (ALD) treated with erucic acid (22:1)-ric h triglycerides ('Lorenzo's oil'). To investigate possible alterations of biophysical membrane properties, we measured platelet membrane ani sotropy, which is inversely related to membrane fluidity, in 16 patien ts with and in 3 patients without treatment. In patients on treatment, platelet membrane anisotropy was significantly decreased. Additionall y, we found increased platelet concentrations of 22:1 and compromised in vitro platelet aggregation response. The decrease of platelet membr ane anisotropy is probably a main cause of bleeding diathesis. Long-te rm haematological side-effects must be considered in ALD patients trea ted with Lorenzo's oil.