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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 [1]
Complications:
- myelofibrosis
Management:
- platelet transfusions for active bleeding as needed
- epsilon-aminocaproic acid for active bleeding as needed [1]
Related
beta-thromboglobulin 1 (TGB1, THBGB1)
platelet factor 4; PF-4; C-X-C motif chemokine 4; iroplact; oncostatin-A (PF4, CXCL4, SCYB4)
General
platelet disorder; thromboasthenia
References
- Medical Knowledge Self Assessment Program (MKSAP) 11, 17, 18.
American College of Physicians, Philadelphia 1998, 2015, 2018.