R. Barmaki Pour et al., Enhanced lymphocyte proliferation in patients with adrenoleukodystrophy treated with erucic acid (22 : 1)-rich triglycerides, J INH MET D, 23(2), 2000, pp. 113-119
Lymphocytopenia and depression of natural killer cells have been observed i
n patients with adrenoleukodystrophy (ALD) treated with glycerol trioleate
and glycerol trierucate ('Lorenzo's oil'). To investigate possible alterati
ons of cellular immunoreactivity, we measured lymphocyte proliferation in r
esponse to mitogens (PHA, Con A, PWM, OKT3) in 27 patients on treatment and
in 14 patients without treatment. In patients on treatment, lymphocyte pro
liferation in response to the mitogens PHA and Con A was significantly high
er than in patients without treatment. Lymphocyte proliferation in patients
without treatment was comparable to that of normal control lymphocytes. Ad
ditionally, we found increased concentrations of erucic acid, C-22:1, in ly
mphocytes from patients with treatment. The enhanced proliferation of lymph
ocytes in response to mitogens is an indication of increased reactivity of
cellular immunity to unspecific immunological stimuli. Long-term side-effec
ts on cellular immunoreactivity have to be considered in ALD patients treat
ed with Lorenzo's oil.