Table 1.
Characteristics of patients with CVST according to Jak2V617F mutation status
| JAK2V617F-negative CVST (n = 207) | JAK2V617F-positive CVST (n = 9) | P value | |
|---|---|---|---|
| Age, y | 39 [28-51] | 42 [24-75] | .45 |
| Male/female | 70/137 | 0/9 | .033 |
| BMI (kg/m2) | 25.6 [22.5-29] | 23.6 [21.5 27.2] | .27 |
| Mode of onset | |||
| Acute | 73/207 (35%) | 3/9 (33%) | .90 |
| Subacute | 118/207 (57%) | 6/9 (67%) | .56 |
| Chronic | 16/207 (8%) | 0/9 (0%) | 1 |
| Clinical findings | |||
| Isolated intracranial hypertension | 105/207 (50.7%) | 6/9 (66.6%) | .5 |
| Focal deficits | 87/207 (42%) | 2/9 (22.2%) | .31 |
| Encephalopathy | 13/207 (6.2%) | 1/9 (11.1%) | .46 |
| Acquired thrombophilia | |||
| Antiphospholipid syndrome | 1/81 (12%) | 0/9 (0%) | 1 |
| Hormonal (OC and/or pregnancy∗) | 101/207 (48.7%) | 4/9 (44.4%) | 1 |
| Systemic disorders† | 21/207 (10.1%) | 0/9 (0%) | .60 |
| Extracerebral neoplasia | 6/207 (2.8%) | 0/9 (0%) | 1 |
| Inherited thrombophilia | |||
| Protein C deficiency | 3/192 (1.6%) | 0/9 (0%) | 1 |
| Protein S deficiency | 8/191 (4.2%) | 0/9 (0%) | 1 |
| Antithrombin deficiency | 3/187 (1.6%) | 0/9 (0%) | 1 |
| Factor V Leiden | 15/142 (11.2%) | 0/6 (0%) | .6 |
| Factor II G20210A | 20/167 (11.9%) | 1/9 (11.1%) | .93 |
| Local risk factors‡ | 30/207 (14.4%) | 1/9 (11.1%) | 1 |
| Thrombosed sinuses/veins | |||
| SSS | 96/207 (46.3%) | 6/9 (66.6%) | .31 |
| Straight sinus | 26/207 (12.5%) | 3/9 (33.3%) | .10 |
| Lateral sinus | 119/207 (57.4%) | 6/9 (66.6%) | .73 |
| Deep venous system | 13/207 (6.2%) | 1/9 (11.1%) | .45 |
| Cortical veins | 86/207 (41.5%) | 5/9 (55.5%) | .49 |
| Number of sinuses involved | 2 [1-3] | 3 [1.5-4] | .23 |
| Combined sinus/vein | 74/207 (35.7%) | 6/9 (66.6%) | .079 |
| Parenchymal lesions | 78/207 (37.6%) | 4/9 (44.4%) | .73 |
| Ischemic | 26/78 (33.3%) | 2/4 (50%) | .60 |
| Hemorrhagic | 27/78 (34.6%) | 1/4 (25%) | 1 |
| Both | 25/78 (32%) | 1/4 (25%) | 1 |
| Biological findings | |||
| Hemoglobin, g/dL | 13.7 [12.7-15] | 14.1 [13.3-15.5] | .45 |
| Hematocrit, % | 41 [38.5-44] | 41.7 [40-53] | .23 |
| WBC count, ×109/L | 9 [7-11.3] | 13.3 [8.4-19.4] | .062 |
| Neutrophil count, ×109/L | 6.4 [4.5-8.7] | 8 [5.3-11.1] | .055 |
| Platelet count, ×109/L | 260 [208-317] | 319 [222-443] | .11 |
| D-dimers, ng/mL | 968 [553-1545] | 1205 [493-2043] | .91 |
| CRP | 12 [6-28] | 16 [7.5-35.5] | .45 |
| Fibrinogen, g/L | 4 [3.4-4.7] | 4.1 [3.5-4.9] | .74 |
| MPO (ng/mL) | 14.7 [9-22.4] | 19.7 [15-26] | .094 |
| SII index | 923 [531-1692] | 1664 [1066-1989] | .019 |
| Outcome (mRS, 3 mo follow-up) | |||
| <2 | 163/190 (85.7%) | 7/9 (77.7%) | .62 |
| ≥2 | 21/190 (11%) | 1/9 (11.1%) | 1 |
| Death | 6/190 (3.1%) | 1/9 (11.1%) | .28 |
Results are expressed as median [IQR, 25-75] or n (percentage,%).
BMI, body mass index; CRP, C-reactive protein; mRS, modified Rankin Scale; OC, estrogen contraceptive; WBC, white blood cell.
∗
3 cases of pregnancy-related CVST.
†
Systemic disorders include Behçet disease, rheumatologic or connective tissue disease, vasculitis, and sarcoidosis.
‡
Local risk factors include trauma, arteriovenous malformation, paracranial infection. Quantitative data were compared using Mann-Whitney t test. Qualitative data were analyzed by χ2 test.