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Platelet and coagulation studies in Ehlers‐Danlos syndrome
Author(s) -
ANSTEY A.,
MAYNE K.,
WINTER M.,
PETTE J.,
POPE F.M.
Publication year - 1991
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/j.1365-2133.1991.tb06063.x
Subject(s) - ehlers–danlos syndrome , medicine , bleeding diathesis , platelet , factor xiii , coagulation , surgery , gastroenterology , fibrinogen
Summary Fifty‐one patients with Ehlers‐Danlos syndrome were investigated for abnormalities of platelets and coagulation, Thirty‐eight were examined prospectively and 13 retrospectively. A bleeding history was taken from all patients: only four (8%) gave no history of a bruising or bleeding tendency. Nine patients four (18%) had significant haemostatic abnormalities of whom four (8%) had a platelet release defect, three (6%) had a factor XI deficiency and two (4%) had a factor XIII deficiency. Additionally 16 patients (31%) had mild abnormalities of uncertain significance of whom four (8%) had prolonged bleeding times (three in association with platelet aggregation abnormalities 13 (26%) had platelet aggregation abnormalities and two had a positive Hess test. Twenty‐four patients (47%) had normal tests for haemostasis of whom 20 (39%) had a bleeding diathesis and four (8%) had no such tendency. Results were analysed according to the type of Ehlers‐Danlos syndrome, but there was no pattern to the abnormalities. The high frequency of a bleeding tendency in Ehlers‐Danlos patients with normal tests for haemostasis (83%) supports the conventional explanation for this clinical feature, that defects in the structural integrity of skin and blood vessels lead to easy bruising.