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Recurrent Venous Thrombosis in Ehlers-Danlos Syndrome Type III: An Atypical Manifestation

key information

source: BMJ case reports

year: 2013

authors: Jiménez-Encarnación E, Vilá LM

summary/abstract:

Ehlers-Danlos syndrome (EDS) comprises a group of hereditary connective tissue disorders in which collagen synthesis and fibrogenesis are impaired. Patients with EDS type III have a bleeding tendency manifested by ecchymoses and haematomas. However, thrombotic events are rare in this entity. Herein, we present a 48-year-old Hispanic man with EDS type III who had recurrent cephalic vein thrombophlebitis and thrombosis, and brachial vein thrombosis. Tests for hypercoagulable disorders including antithrombin III activity, protein C activity, protein S activity, anticardiolipin antibodies, homocysteine levels, factor V Leiden mutation and prothrombin gene mutation were negative. The patient required long-term anticoagulation with warfarin. After 3 years follow-up, he did not present further thrombotic events. Clinicians should be aware that patients with EDS might be at risk for hypercoagulable disorders.

organization: University of Puerto Rico Medical Sciences Campus

DOI: 10.1136/bcr-2013-008922

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