Discontinuation of fucose therapy in LADII causes rapid loss of selectin ligands and rise of leukocyte counts.

Abstract:

: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.

journal_name

Blood

journal_title

Blood

authors

Lühn K,Marquardt T,Harms E,Vestweber D

doi

10.1182/blood.v97.1.330

subject

Has Abstract

pub_date

2001-01-01 00:00:00

pages

330-2

issue

1

eissn

0006-4971

issn

1528-0020

journal_volume

97

pub_type

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