A new genetic isolate of gray platelet syndrome (GPS): clinical, cellular, and hematologic characteristics

TC Falik-Zaccai, Y Anikster, CE Rivera… - Molecular genetics and …, 2001 - Elsevier
Gray platelet syndrome (GPS) is a disorder characterized by thrombocytopenia and large
platelets that lack α granules and their contents. We describe two siblings with GPS who are
members of a Moslem Bedouin genetic isolate. The children, an 8-year-old girl and a 5-year-
old boy, had characteristic pale blue platelets lacking α granules on electron microscopy.
Platelet aggregation studies were normal. The girl underwent a bone marrow aspiration and
biopsy which showed mild myelofibrosis and extensive emperipolesis, ie, the passage of …