Enhanced lymphocyte proliferation in patients with adrenoleukodystrophy treated with erucic acid (22 : 1)-rich triglycerides

Citation
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
Citations number
13
Categorie Soggetti
Endocrinology, Nutrition & Metabolism
Journal title
JOURNAL OF INHERITED METABOLIC DISEASE
ISSN journal
01418955 → ACNP
Volume
23
Issue
2
Year of publication
2000
Pages
113 - 119
Database
ISI
SICI code
0141-8955(200003)23:2<113:ELPIPW>2.0.ZU;2-T
Abstract
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.