Abstract
A case of type IV Ehlers-Danlos syndrome with a partial platelet δ-storage pool disease is reported. The diagnosis of Ehlers-Danlos was clinical. The platelet-dense granule deficiency was determined by ultrastructural platelet morphology. Dense bodies were decreased in number, and most showed loss or fragmentation of electron-dense material. Aggregation studies revealed a retarded response to ristocetin and arachidonic acid, which was corrected with desmopressin acetate-DDAVP.
Original language | English |
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Pages (from-to) | 47-50 |
Journal | Annals of Hematology |
Volume | 77 |
DOIs | |
Publication status | Published - 1 Jul 1998 |
Keywords
- Dense granule deficiency
- Ehlers-Danlos syndrome
- δ-storage pool disease