Diagnosis of Bernard-Soulier syndrome and Glanzmann's thrombasthenia with a monoclonal assay on whole blood.

A C T Two hereditary platelet disorders, Ber- nard-Soulier syndrome and Glanzmann's thrombasthenia, are characterized by selective deficiencies of platelet membrane glycoproteins.Murine monoclonal antibodies were developed against platelet membrane glycoprotein Ib and against the glycoprotein Ilb/Il1a complex.A rapid whole blood assay for the deficiency of these glycoproteins was developed and used to study whole blood samples from six patients with Glanzmann's thrombasthenia and three patients with Ber- nard-Soulier syndrome.Patients with type I and type II Glanzmann's thrombasthenia were easily detectable with this assay.This permits the diagnosis of these disorders on 200 Ml of whole blood within 2 h of blood sampling.Dr. Montgomery is an Established Investigator of the American Heart Association, 82-198.Dr. Kunicki is the re- cipient of a New Investigator Award (HL 27638) from the National Heart, Lung, and Blood Institute.Address all cor- respondence to Dr. Montgomery.