TY - JOUR
T1 - Type IV Ehlers-Danlos syndrome with platelet δ-storage pool disease
AU - Español, I.
AU - Hernández, A.
AU - Pujol, R. M.
AU - Urrutia, T.
AU - Pujol-Moix, N.
PY - 1998/7/1
Y1 - 1998/7/1
N2 - 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.
AB - 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.
KW - Dense granule deficiency
KW - Ehlers-Danlos syndrome
KW - δ-storage pool disease
U2 - https://doi.org/10.1007/s002770050410
DO - https://doi.org/10.1007/s002770050410
M3 - Article
VL - 77
SP - 47
EP - 50
JO - Annals of Hematology
JF - Annals of Hematology
SN - 0939-5555
ER -