Table 2.
Clinical Manifestations of ET Patients with CVST
| No. | Gender/Age (y) | Types of Onset | Neurological Clinical Features | Splenomegaly | JAK2 V617F Mutation | Bone Marrow Biopsy | Cerebral Parenchymal Lesions | Site of CVST | IPSET-Thrombosis Score | Treatment |
|---|---|---|---|---|---|---|---|---|---|---|
| 1 | M/71 | Sub-acute | Headache, papilledema, nausea/vomiting, impaired cognition, mental abnormalities | Yes | Positive | Megakaryocytic hyperplasia | CVI, ICH | SSS, (B)TS, (B) SiS, SS,CS | 3 | Aspirin, LMWH, warfarin |
| 2 | F/22 | Acute | Headache, papilledema | No | Negative | Megakaryocytic hyperplasia | ICH in right frontal and parietal | SSS, (B)TS, (B) SiS | 5 | Aspirin, clopidogrel, LMWH, warfarin, IT, hydroxyurea |
| 3 | F/33 | Chronic | Headache, papilledema, blurred vision | Yes | Positive | Myeloproliferative activity | None | SSS, SS, (B) TS, CS | 4 | Aspirin, LMWH, warfarin, hydroxyurea |
| 4 | F/34 | Acute | Headache, nausea/vomiting, conscious disturbance, papilledema | Yes | Positive | Megakaryocytic hyperplasia | Bilateral thalamus CVI | SSS, (L)TS, (L) SiS | 4 | Aspirin, LMWH, warfarin, IFN-a |
| 5 | M/43 | Acute | Headache, papilledema, limbs numbness, neck pain, seizure | Yes | Positive | Megakaryocytic hyperplasia | CVI in the right hemisphere | SSS, (R) TS, (R) SiS | 3 | LMWH, warfarin, IT, ET, hydroxyurea |
| 6 | M/20 | Chronic | Headache, blurred vision | Yes | Positive | Megakaryocytic hyperplasia | Bilateral centrum semiovale and left parietooccipital CI | SSS, SS, CV | 5 | LMWH, warfarin, IFN-a, hydroxyurea |
| 7 | F/28 | Sub-acute | Headache, papilledema, nausea/vomiting, blurred vision, visual field defect | No | Positive | Megakaryocytic hyperplasia | None | SSS, SS, (L)TS, (L) SiS, (L) JV | 4 | Aspirin, LMWH, warfarin, hydroxyurea, IFN-a, corticosteroid |
| 8 | M/23 | Sub-acute | Headache, papilledema, nausea/vomiting, blurred vision, neck pain | Yes | Positive | Megakaryocytic hyperplasia | None | SSS, (B)TS, (B) SiS | 4 | Clopidogrel, LMWH, dabigatran, IFN-a, hydroxyurea |
| 9 | F/64 | Chronic | Headache, papilledema, visual loss | No | Positive | NA | None | SSS, (B)TS, (B)SiS | 5 | Warfarin, hydroxyurea |
| 10 | F/55 | Chronic | Headache, papilledema, nausea/vomiting, blurred vision | Yes | Positive | Active myeloproliferative activity | None | (R)TS, (R) SiS | 4 | Aspirin, LMWH, warfarin, hydroxyurea |
| 11 | M/25 | Chronic | Headache, papilledema, tinnitus, dizziness, loss of appetite, amaurosis fugax | No | Positive | Megakaryocytic hyperplasia | Left frontal CI | SSS, (B)TS, (B) SiS, (B) JV | 4 | Aspirin, LMWH, warfarin, corticosteroid, cyclophosphamide |
| 12 | F/17 | Acute | Headache, papilledema, nausea/vomiting, weakness of upper limb, dysarthria, blurred vision, seizure | Yes | Positive | Megakaryocytic hyperplasia | HCVI in the left partial | SSS, (B) SiS, (B) JV | 4 | Aspirin, LMWH, warfarin, ET |
| 13 | F/39 | Acute | Blurred vision/visual loss, headache, tinnitus, conscious disturbance, aphasia and hemiplegia, seizure | No | Positive | NA | HCVI in the left hemisphere | SSS, CV | 4 | LMWH, warfarin, ET, hydroxyurea |
| 14 | F/28 | Acute | Headache, nausea/vomiting, seizure | No | Positive | NA | Bilateral frontal CI | (L) TS, (L) SiS | 4 | LMWH, dabigatran, IT |
| 15 | F/25 | Chronic | Headache, papilledema, dizziness, nausea, blurred vision, metamorphopsia, tinnitus | No | Positive | NA | None | (B) TS, (B) SiS | 4 | Aspirin, LMWH, warfarin, cyclophosphamide, IT, corticosteroid |
| 16 | M/50 | Acute | Headache, papilledema, nausea/vomiting | No | Positive | Myeloproliferative activity | None | (L) TS, (L) SiS | 4 | Aspirin, warfarin, ET, hydroxyurea, stenting |
| 17 | M/31 | Chronic | Headache, papilledema, blurred vision/visual loss | No | Positive | Megakaryocytic hyperplasia | None | SSS, (R)TS, (R) SiS, (R) JV | 4 | Aspirin, LMWH, warfarin, corticosteroid, hydroxyurea |
| 18 | F/42 | Acute | Headache, papilledema, nausea, seizure, aphasia and hemiplegia | No | Positive | Megakaryocytic hyperplasia | Bilateral parietal CVI | SSS, ISS, (L) SiS, SS, CS, (B) CV, (B) TS, (L) JV | 4 | Aspirin, LMWH, dabigatran, IT |
| 19 | F/35 | Acute | Headache, papilledema, nausea/vomiting, seizure | Yes | Negative | NA | HCVI in the left hemisphere | SSS, (L)TS, (L) SiS | 2 | Aspirin, warfarin, hydroxyurea, decompressive craniectomy |
| 20 | M/53 | Acute | Headache, papilledema, conscious disturbance, blurred vision | Yes | Positive | Megakaryocytic hyperplasia | None | SSS, (R)TS, (R) SiS | 4 | Aspirin, warfarin, hydroxyurea |
| 21 | F/43 | Sub-acute | Headache, papilledema, tinnitus, hearing loss, dizziness, blurred vision, edema of eyelids | No | Positive | Megakaryocytic hyperplasia | Left frontal CI | (L) TS, (L) SiS, (L) JV | 4 | Aspirin, warfarin, IFN-α |
| 22 | F/33 | Acute | Headache, nausea/vomiting | No | Positive | Myeloproliferative activity | None | SSS, (B) TS, (B) SiS | 4 | Aspirin, LMWH, warfarin, IFN-α |
| 23 | M/52 | Acute | Headache, nausea/vomiting, papilledema, conscious disturbance, hemiplegia | No | Positive | Megakaryocytic hyperplasia | HCVI in the left frontal lobe | SSS, (L) SiS | 5 | LMWH, warfarin, hydroxyurea |
Abbreviations: ET, essential thrombocythemia; CVST, cerebral venous sinus thrombosis; NA, not available; CI, cerebral infarction; CVI, cerebral venous infarction; HCVI, hemorrhagic cerebral venous infarction; ICH, intracranial hemorrhage; B, bilateral; L, left; R, right; SSS, superior sagittal sinus; ISS, inferior sagittal sinus; SiS, sigmoid sinus; TS, transverse sinus; SS, straight sinus; CS, confluence of sinuses; JV, jugular vein; CV, cortical vein; LMWH, low molecular weight heparin; CS, corticosteroid; IT, intrasinus thrombolysis; ET, endovascular thrombectomy.