A sickle cell disease patient with dural venous sinus thrombosis: a case report and literature review. (4th May 2019)

• Record Type: Journal Article
• Authors: Wang, Michael K.; Shergill, Ravi; Jefkins, Matthew; Cheung, Jason
• Is Part Of: Hemoglobin. Volume 43:Number 3(2019)
• Page Start: 193
• Page End: 197
• Publication Date: 2019-05-04
• Subjects: Dural venous sinus thrombosis (DVST) -- hypercoagulability -- internal jugular vein thrombosis -- sickle cell disease; Hemoglobinopathy -- Periodicals; Hemoglobin -- Periodicals; Hematology -- Periodicals; Thalassemia -- Periodicals; Blood -- Diseases -- Periodicals; 612.1111
• DOI: 10.1080/03630269.2019.1651734
• Languages: English
• ISSNs: 0363-0269

Abstract

Abstract: Dural venous sinus thrombosis (DVST) is a rare disease associated with hypercoagulable states. Patients with sickle cell disease are known to be prothrombotic. We report a case of DVST presenting with anterior neck and facial pain in a 24-year-old female with sickle cell disease, found to have extensive thrombotic disease involving the internal jugular vein. A literature review of DVST in sickle cell disease consisting of 14 case reports was summarized. Headache was a presenting feature in two-thirds of patients. Nine cases were associated with vaso-occlusive crisis (VOC), transfusion, or acute respiratory illness. Most patients were treated with anticoagulation therapy. Over three-quarters either died or suffered from a serious neurological complication, including stroke, seizure, coma, or elevated intracranial pressure. Given its association with life-threatening complications, DVST should be considered when patients with sickle cell disease present with a VOC, especially in the context of headache or neurological deficits.