gray platelet syndrome

Pathology

• congenital platelet defects
• absence of alpha granules
• low beta-thromboglobulin
• low platelet factor 4

Clinical manifestations

• moderate rather than severe bleeding disorder

Complications

• myelofibrosis

Management

• platelet transfusions for active bleeding as needed
• epsilon-aminocaproic acid for active bleeding as needed

More general terms

• platelet disorder; thromboasthenia

Additional terms

• beta-thromboglobulin 1 (TGB1, THBGB1)
• platelet factor 4; PF-4; C-X-C motif chemokine 4; iroplact; oncostatin-A (PF4, CXCL4, SCYB4)