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 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.
Stöckler
S
S
Department of Neuropediatrics, Children's Hospital University of Göttingen, Germany.
Opper
C
C
Greinacher
A
A
Hunneman
D H
DH
Korenke
G C
GC
Unkrig
C J
CJ
Hanefeld
F
F
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*
D004883:Erucic Acids; D014280:Triglycerides; C049811:erucic acid