A new lysosomal storage disorder resembling Morquio syndrome in sibs

► We report two males sibs presenting with a storage disorder resembling Morquio disease. ► No lysosomal enzyme defect could be identified. ► Blood smear showed in both children normal lymphocytes but abnormal coarse inclusions in eosinophils, mild Alder anomaly in polymorphonuclears. Granulations were discretely metachromatic, in favor of a storage disease. ► Transmission could be autosomal recessive or X-linked.