Massive cerebral venous thrombosis due to vaccine-induced immune thrombotic thrombocytopenia

Italy, 1 November 2021, Bonato et al, Haematolgica

Summary

We describe an Italian case of severe VITT-related cerebral venous thrombosis (CVT) and bi-hemispheric hemorrhage, which was treated with argatroban, intravenous immunoglobulin (IVIG) and corticosteroids.

A previously healthy 26-year-old female presented to the emergency department 14 days after the first injection of ChAdOx1 nCoV-19 vaccine with a headache nonresponsive to anti-inflammatory drugs. On admission, she had right-sided weakness and visual disturbances. She has been on combined (estrogen-progestogen) contraceptives for more than 10 years but her past medical history was otherwise unremarkable and there was no prior exposure to heparin.

While general examination and vital signs were normal, neurological examination found a severe right-sided weakness but no visual field defects. Computerised tomography (CT) scan at admission showed a hyperdense rectus sinus and vein of Galen (Figure 1A). Magnetic resonance imaging (MRI) venography showed multifocal venous thrombosis with bilateral occlusion of parietal cortical veins, straight sinus, vein of Galen, internal cerebral veins and inferior sagittal sinus. Transverse sinuses were also partially involved but still patent (Figure 1B). At the right parietal and left frontoparietal lobes an extensive venous infarction with hemorrhagic transformation was present (Figure 1C). D-dimer was dramatically raised to 12,204 mg/L (reference value <500 mg/L) and the platelet count was 134×109/L. Given her recent exposure to ChAdOx1 nCoV-19 and clinical presentation, she was first treated with fondaparinux (5 mg subcutaneously) and admitted her to the intensive care unit. Her clinical condition rapidly deteriorated with decreased consciousness, right-sided hemiplegia and complete Balint syndrome. Owing to the possible need for a sudden decompressive neurosurgical intervention, anticoagulation with fondaparinux was replaced by the short-acting drug argatroban

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Neuroradiological findings at baseline and follow-up. (A) baseline computerised tomography (CT) (at admission) shows hyperdense rectus sinus and vein of Galen as signs of thrombosis (arrow). (B) Magnetic resonance imaging (MRI) examination (day 1) confirms complete occlusion of the rectus sinus, vein of Galen, right internal cerebral vein (arrow) and frontoparietal cortical veins on both sides (arrow head) on venous angiography. (C) On coronal T2-FLAIR images extensive venous infarctions with hemorrhagic transformation can be seen in right parietal (arrow) and left frontoparietal (arrow head) regions. (D) On follow-up CT (day 6) rectus sinus and vein of Galen show normal density (arrow) with oedema in brain tissue on both hemispheres (arrow head). Follow-up MRI (day 7) shows (E) restored venous flow in the rectus sinus and the vein of Galen (arrow); right internal cerebral vein and bilateral frontoparietal cortical veins are still occluded. (F) On T2 weighted images the large bilateral venous infarctions are still visible with hemorrhagic transformation in pre- and postcentral gyrus in the left side (arrow).

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