Glueck, Thomas and Mueller-Ladner, Ulf and Speicher, A. and Pickenpack, A. and Roeckelein, G. and Schoelmerich, Juergen (2001) Lethal sinus vein thrombosis in a patient with mixed connective tissue disease and antiphospholipid syndrome. MEDIZINISCHE KLINIK, 96 (6). pp. 361-364. ISSN 0723-5003, 1615-6722
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Background: The antiphospholipid (Huges) syndrome is a complication of connective tissue diseases characterized by thromboembolic occlusions of arterial and venous blood vessels. Case Report: At the age of 13, the patient developed connective tissue disease with arthritis and myositis. The course of her disease was characterized by frequent relapses despite immunosuppressive treatment. She developed deep venous thrombosis of her right leg as a manifestation of secondary antiphospholipid antibody syndrome at the age of 15 and was subsequently started on oral anticoagulation therapy. Approximately 10 months later, however, she decided to try alternative medicine and stopped both anticoagulation and immunosuppressive therapy. Only after 4 weeks she developed seizures followed by respiratory arrest with the need for cardiopulmonary resuscitation. Despite intensive care she died 2 days later with the signs of severe cerebral edema causing herniation of the brainstem. Autopsy confirmed the diagnosis of severe edema of the brain as a result of extensive thrombosis of all sinus veins. Conclusion: A complete sinus vein thrombosis is a rare manifestation of antiphospholipid antibody syndrome. The lethal thrombosis in this case occurred during a period of reactive hypercoagulability after termination of immunosuppressive and/or anticoagulation therapy. This case report underlines the need for long-term anticoagulation in patients with the antiphospholipid syndrome.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | SYSTEMIC LUPUS-ERYTHEMATOSUS; CEREBRAL VENOUS THROMBOSIS; ANTICARDIOLIPIN ANTIBODIES; CARDIOLIPIN; INFECTIONS; YOUNG; mixed connective tissue disease; antiphospholipid syndrome; sinus vein thrombosis; seizures |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Innere Medizin I |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 17 Jan 2022 09:48 |
| Last Modified: | 17 Jan 2022 09:48 |
| URI: | https://pred.uni-regensburg.de/id/eprint/41340 |
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