GRAY PLATELET SYNDROME WITH SPLENOMEGALY AND SIGNS OF EXTRAMEDULLARY HEMATOPOIESIS - A CASE-REPORT WITH REVIEW OF THE LITERATURE

Gray platelet syndrome (GPS) is a rare bleeding disorder characterized by thrombocytopenia, agranular gray platelets in blood films, and alm ost total absence of platelet alpha-granules and their constituents. W e describe here a rare case of GPS with myelofibrosis and splenomegaly indicating extramedullary hematopoiesis. Splenectomy was followed by normalization of platelet count but the bleeding diathesis continued. Based on a follow-up of more than 15 years, the slight myelofibrosis i n our patient seems to be non-progressive. We also summarize the major clinical and laboratory features of previously published cases of GPS in order to obtain more comprehensive understanding of this rare diso rder. From the clinical point of view, the bleeding tendency in this s yndrome generally varies from mild to moderate, and no specific treatm ent is usually needed. Careful examination of peripheral blood films i s necessary for the diagnosis of this rare syndrome. (C) 1994 Wiley-Li ss, Inc.