Characteristics and prognostic scores associated with the JAK2V617F mutation in 104 patients with Budd-Chiari syndrome
| BCS patients . | Without JAK2V617F, n = 57 (median [Q1-Q3]) . | With JAK2 V617F, n = 47 (median [Q1-Q3]) . | P . |
|---|---|---|---|
| Age, y | 40 [33-46] | 46 [35-51] | .2 |
| Males, % | 24 (42.1%) | 11 (23.4%) | .061 |
| Hemoglobin, g/dL | 13 [11.6-14.2] | 14.6 [13-16.1] | .003 |
| Hematocrit, % | 39.5 [34.7-46] | 46.4 [41.8-49.5] | .002 |
| MCV, fL | 88 [83-91.6] | 84 [79-86.8] | .003 |
| WBC count, ×109/L | 6.7 [5-9] | 9.85 [6.58-14.2] | <.001 |
| ANC, ×109/L | 4.03 [3.1-5.47] | 7.35 [4.5-10.1] | .004 |
| Platelet count, ×109/L | 185 [117-269] | 352.5 [284.5-456] | <.001 |
| Spleen size BCM, cm | 0 [0-1] | 1 [0-5] | .03 |
| Serum EPO, mU/mL | 22.8 [15.4-46.7] | 13.25 [8-23.1] | .008 |
| Measured RCM, mL/kg | 25.6 [21.8-29.1] | 31.15 [26.8-35.2] | .007 |
| Predicted RCM, mL/kg | 24 [21.25-28.22] | 23.45 [23-26.5] | .8 |
| RCM more then 125% of predicted | 0/18 (0%) | 18/30 (60.0%) | <.001 |
| AST, ULN | 38 [26.25-7] | 78.5 [39-258] | .002 |
| ALT, ULN | 38 [21.5-91.5] | 80 [33.2-239.8] | .015 |
| Serum bilirubin, μmol/L | 23.97 [16-42.3] | 43 [22-61.2] | .025 |
| Serum creatinine, μmol/L | 79 [61.9-86] | 75 [60-89] | .56 |
| Serum albumin, g/dL | 38 [31.5-41] | 33.5 [29.9-41] | .38 |
| Factor V, % | 74 [51-97] | 45 [30-59.5] | <.001 |
| Prognostic scores | |||
| Child-Pugh score | 9 [7-10.75] | 10 [9-11] | .004 |
| Clichy prognostic index | 5.63 [5.12-6.63] | 6.51 [5.83-7.4] | .006 |
| Rotterdam BCS score | 2.37 [1.37-2.43] | 2.47 [2.36-2.59] | .002 |
| BCS patients . | Without JAK2V617F, n = 57 (median [Q1-Q3]) . | With JAK2 V617F, n = 47 (median [Q1-Q3]) . | P . |
|---|---|---|---|
| Age, y | 40 [33-46] | 46 [35-51] | .2 |
| Males, % | 24 (42.1%) | 11 (23.4%) | .061 |
| Hemoglobin, g/dL | 13 [11.6-14.2] | 14.6 [13-16.1] | .003 |
| Hematocrit, % | 39.5 [34.7-46] | 46.4 [41.8-49.5] | .002 |
| MCV, fL | 88 [83-91.6] | 84 [79-86.8] | .003 |
| WBC count, ×109/L | 6.7 [5-9] | 9.85 [6.58-14.2] | <.001 |
| ANC, ×109/L | 4.03 [3.1-5.47] | 7.35 [4.5-10.1] | .004 |
| Platelet count, ×109/L | 185 [117-269] | 352.5 [284.5-456] | <.001 |
| Spleen size BCM, cm | 0 [0-1] | 1 [0-5] | .03 |
| Serum EPO, mU/mL | 22.8 [15.4-46.7] | 13.25 [8-23.1] | .008 |
| Measured RCM, mL/kg | 25.6 [21.8-29.1] | 31.15 [26.8-35.2] | .007 |
| Predicted RCM, mL/kg | 24 [21.25-28.22] | 23.45 [23-26.5] | .8 |
| RCM more then 125% of predicted | 0/18 (0%) | 18/30 (60.0%) | <.001 |
| AST, ULN | 38 [26.25-7] | 78.5 [39-258] | .002 |
| ALT, ULN | 38 [21.5-91.5] | 80 [33.2-239.8] | .015 |
| Serum bilirubin, μmol/L | 23.97 [16-42.3] | 43 [22-61.2] | .025 |
| Serum creatinine, μmol/L | 79 [61.9-86] | 75 [60-89] | .56 |
| Serum albumin, g/dL | 38 [31.5-41] | 33.5 [29.9-41] | .38 |
| Factor V, % | 74 [51-97] | 45 [30-59.5] | <.001 |
| Prognostic scores | |||
| Child-Pugh score | 9 [7-10.75] | 10 [9-11] | .004 |
| Clichy prognostic index | 5.63 [5.12-6.63] | 6.51 [5.83-7.4] | .006 |
| Rotterdam BCS score | 2.37 [1.37-2.43] | 2.47 [2.36-2.59] | .002 |
MCV indicates mean cell volume; WBC, white blood cell; ANC, absolute neutrophil count; BCM, below costal margin; and RCM, red cell mass.