MORPHOLOGIC AND HEMATOLOGIC CHARACTERISTICS OF STORAGE POOL DEFICIENCY IN BEIGE RATS (CHEDIAK-HIGASHI-SYNDROME OF RATS)

Characterization of beige rats as having a platelet storage pool defic iency (SPD) was undertaken. Platelets from beige rats, an animal model of Chediak-Higashi syndrome (CHS), completely lacked the ability to a ggregate when stimulated with high dosages of collagen (50 mu g/ml), a nd lacked secondary aggregation induced by adenosine diphosphate (ADP) . Concentrations of ADP, ATP, and serotonin in the platelets of beige rats were significantly lower than those of control rats. However, pla telet count remained within normal values. Electron microscopy reveale d that platelets had fewer dense granules, whereas other organelles ha d normal structure. This morphologic and functional evidence confirms that platelets of beige rats have the typical characteristics of SPD.