Type IV Ehlers-Danlos syndrome with platelet δ-storage pool disease

I. Español, A. Hernández, R. M. Pujol, T. Urrutia, N. Pujol-Moix

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11 Citations (Scopus)


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 languageEnglish
Pages (from-to)47-50
JournalAnnals of Hematology
Publication statusPublished - 1 Jul 1998


  • Dense granule deficiency
  • Ehlers-Danlos syndrome
  • δ-storage pool disease


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