ristocetin and Spherocytosis--Hereditary

Thrombocyte function was studied in seven splenectomized patients and one unsplenectomized patient with hereditary or congenital spherocytosis (HS). Adenosine diphosphate (ADP) concentrations, which trigger the aggregation of control thrombocytes, induced only a release reaction in thrombocytes from six of the HS patients. Collagen-induced thrombocyte aggregation was also diminished in two patients, and epinephrine-induced thrombocyte aggregation in one patient. Two patients whose thrombocytes responded normally to ADP, collagen and epinephrine, were identical twins with spherocytosis of equivocal hereditary. Ristocetin-induced aggregation was normal in all the patients studied. Xanthines (pentoxifylline, theophylline) had no effect on the aggregation of thrombocytes from HS patients or controls. Half-life of thrombocytes was normal in the two splenectomized patients studied. The results demonstrate that not only red cells but also thrombocytes are defective in HS.

Topics: Adenosine Diphosphate; Adult; Chromium Radioisotopes; Collagen; Epinephrine; Female; Humans; Male; Middle Aged; Pentoxifylline; Platelet Aggregation; Ristocetin; Spherocytosis, Hereditary; Splenectomy; Theophylline