Correspondence

Lorenzo's Oil and Lymphocytopenia

N Engl J Med 1994; 330:577February 24, 1994

Article

To the Editor:

Administration of oleic and erucic acids (“Lorenzo's oil”), a widely used therapy for patients with adrenoleukodystrophy or adrenomyeloneuropathy,1,2 is associated with thrombocytopenia3. We studied 15 men with adrenomyeloneuropathy and 3 symptomatic heterozygous women. The patients followed a low-fat diet with restricted intake of C26:0 fatty acid and received a mean (±SD) of 1.28 ±0.14 g of glycerol trioleate oil (Karlshamns, Columbus, Ohio) and 0.23 ±0.02 g of glycerol trierucate oil (Croda Universal, Liverpool, United Kingdom) per kilogram of body weight per day.

Asymptomatic thrombocytopenia developed in five patients (whose platelet counts ranged between 37,000 and 84,000 per cubic millimeter) but was reversed within two to three weeks after erucic acid was omitted, a result in accord with the observations of Aubourg et al. (Sept. 9 issue)1 and Zinkham et al.3. In addition, long-term treatment with Lorenzo's oil (for 24 to 43 months) was associated with lymphocytopenia in these five patients. The absolute numbers of lymphocytes were significantly reduced (mean, 440 per cubic millimeter; range, 10 to 670; P<0.0025). The total white-cell counts and the absolute values for neutrophils and monocytes were within the normal range. Plasma levels of immunoglobulins (IgM, IgG, IgA, and IgE) were normal, and there were no relative reductions in values for T cells, B cells, or natural killer cells according to flow cytometric analysis. The CD4:CD8 ratio was also normal. No cytotoxic antibodies directed at lymphocytes were detected.

Two of the patients had recurrent infections while lymphocytopenic and had anergy on skin testing for delayed hypersensitivity. One patient was admitted to the hospital because of severe enterocolitis with bloody stools, abdominal cramping, and fever, with a markedly reduced lymphocyte count (10 to 40 per cubic millimeter). After oleic and erucic acids were discontinued, lymphocyte counts returned to normal. Interestingly, in all five patients, lymphocytopenia occurred only after more than 24 months of treatment and lymphocyte counts became normal only 6 to 9 weeks after the discontinuation of therapy. The pattern differs from the course of the thrombocytopenia and suggests that there are different mechanisms.

Our observation suggests that the long-term treatment of adrenoleukodystrophy with Lorenzo's oil can induce severe lymphocytopenia with immunosuppression and recurrent infections. Therefore, patients being treated with Lorenzo's oil should be followed closely, with monitoring of lymphocyte counts to prevent infectious complications.

Christoph J. Unkrig, M.D.
Rolf Schroder, M.D.
Rudiger E. Scharf, M.D.
University Hospital, D-53105 Bonn, Germany

3 References

1
Aubourg P, Adamsbaum C, Lavallard-Rousseau M-C, et al. A two-year trial of oleic and erucic acids (“Lorenzo's oil”) as treatment for adrenomyeloneuropathy. N Engl J Med 1993;329:745-752
Full Text | Web of Science | Medline

2
Rizzo WB. Lorenzo's oil -- hope and disappointment. N Engl J Med 1993;329:801-802
Full Text | Web of Science | Medline

3
Zinkham WH, Kickler T, Borel J, Moser HW. Lorenzo's oil and thrombocytopenia in patients with adrenoleukodystrophy. N Engl J Med 1993;328:1126-1127
Full Text | Web of Science | Medline

Author/Editor Response

Dr. Aubourg replies:

To the Editor: We observed no lymphocytopenia in our patients, but rather neutropenia. Recent investigations of the half-life of platelets labeled with indium-111 have demonstrated marked splenic sequestration of platelets in treated patients.

Patrick Aubourg, M.D.
INSERM Unite 342, Paris 75014, France

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