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Blood, Vol. 94 No. 12 (December 15), 1999:
pp. 3976-3985
Correction of Leukocyte Adhesion Deficiency Type II With Oral Fucose
Thorsten Marquardt,
Kerstin Lühn,
Geetha Srikrishna,
Hudson
H. Freeze,
Erik Harms, and
Dietmar Vestweber
From the Klinik und Poliklinik für Kinderheilkunde and the
Institut für Zellbiologie, ZMBE, Universität Münster,
Münster, Germany; the Max-Planck-Institut für
Physiologische und Klinische Forschung, Bad Nauheim, Germany; and The
Burnham Institute, La Jolla, CA.
We describe a simple, noninvasive, and effective therapy for
leukocyte adhesion deficiency type II (LAD II), a rare inherited disorder of fucose metabolism. This disorder leads to an
immunodeficiency caused by the absence of carbohydrate-based selectin
ligands on the surface of neutrophils as well as to severe psychomotor
and mental retardation. The fucosylation defect in LAD II fibroblasts can be corrected by addition of L-fucose to the culture medium. This
prompted us to initiate dietary fucose therapy on a patient with LAD
II. Oral supplementation of fucose in this patient induced the
expression of fucosylated selectin ligands on neutrophils and core
fucosylation of serum glycoproteins. During 9 months of treatment,
infections and fever disappeared, elevated neutrophil counts returned
to normal, and psychomotor capabilities improved.
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