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

Blood. 2001 Jan 1;97(1):330-2. doi: 10.1182/blood.v97.1.330.


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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Fucose / pharmacology
  • Fucose / therapeutic use*
  • Humans
  • Infant
  • Leukocyte Count
  • Leukocyte-Adhesion Deficiency Syndrome / blood
  • Leukocyte-Adhesion Deficiency Syndrome / drug therapy*
  • Ligands
  • Male
  • Neutrophils / chemistry
  • Neutrophils / cytology
  • Selectins / drug effects
  • Selectins / metabolism


  • Ligands
  • Selectins
  • Fucose