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Blood, 1 January 2001, Vol. 97, No. 1, pp. 330-332
BRIEF REPORT
Discontinuation of fucose therapy in LADII causes rapid
loss of selectin ligands and rise of leukocyte counts
Kerstin Lühn,
Thorsten Marquardt,
Erik Harms, and
Dietmar Vestweber
From the Institute of Cell Biology, ZMBE, University of
Münster; Max-Planck-Institute for Physiological and Clinical
Research, Münster, Germany; and Klinik und Poliklinik für
Kinderheilkunde, Münster, Germany.
Leukocyte adhesion deficiency type II (LADII) is a rare
inherited disorder of fucose metabolism. Patients with LADII lack fucosylated glycoconjugates, including the carbohydrate ligands of the
selectins, leading to an immunodeficiency caused by the lack of
selectin-mediated leukocyte-endothelial interactions. A simple and
effective therapy has recently been described for LADII, based on the
administration of oral fucose. Parallel to this treatment the lack of
E- and P-selectin ligands on neutrophils was corrected, and high
peripheral neutrophil counts were reduced to normal levels. This study
reports that discontinuation of this therapy leads to the complete loss
of E-selectin ligands within 3 days and of P-selectin ligands within 7 days. Peripheral neutrophil counts increased parallel to the decrease
of selectin ligands. Selectin ligands reappeared promptly after
resumption of the fucose therapy, demonstrating a causal relationship
between fucose treatment and selectin ligand expression and peripheral
neutrophil counts.
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