Gray platelet syndrome in the elderly.
A 68-year-old male who suffered from thrombocytopenia and mild splenomegaly for 18 years was found to present agranular gray platelets on peripheral blood smear. Bone biopsy revealed a mild, diffuse, reticular fibrosis with no collagen, and electron microscopy of the platelets showed an absence of almost all the alpha-granules. Platelet thrombospondin and fibronectin analysed by SDS-polyacrylamide gel electrophoresis and Rocket immunoelectrophoresis were absent. Follow-up of 4 years showed the same parameters with no evidence of active myeloproliferative or dysmyelopoietic disorders. Hemorrhagic diathesis was limited to ecchymoses and postprostatectomy bleeding, necessitating platelet transfusion. This led us to conclude that our patient probably had a constitutional primary alpha-granule deficiency or gray platelet syndrome. This extremely rare defect has been described in less than 10 patients, all of them very young. Our observation shows that these patients may have a long, uneventful survival.
Hematology Unit, Kaplan Hospital, Rehovot, Israel.
A
A
Berrebi
A
A
Klepfish
D
D
Varon
M
M
Shtalrid
E
E
Vorst
E
E
Nir
J
J
Lahav
D000368:Aged; D001791:Blood Platelet Disorders / Q000151:congenital / Q000175:diagnosis* / Q000473:pathology; D001792:Blood Platelets / Q000473:pathology / Q000648:ultrastructure; D003594:Cytoplasmic Granules / Q000648:ultrastructure; D006801:Humans; D008297:Male; D008854:Microscopy, Electron