Clinical and laboratory features of 187 patients with PMF, stratified by the presence or absence of SRSF2 mutations
| Variable . | All patients (n = 187) . | SRSF2 mutated (n = 32) . | SRSF2 wild-type (n = 155) . | P . |
|---|---|---|---|---|
| Median age, y (range) | 61 (35-81) | 68 (39-81) | 59 (35-81) | .0004‡ |
| Age > 65 y, n (%) | 74 (40) | 20 (63) | 54 (35) | .004‡ |
| Males, % | 117 (63) | 24 (75) | 93 (60) | .11 |
| Median hemoglobin, g/dL (range) | 10 (6-15) | 10 (7-15) | 10 (6-15) | .10 |
| Median leukocytes, × 109/L (range) | 10 (1-147) | 13 (2-46) | 9 (1-147) | .08 |
| Median platelets, × 109/L (range) | 224 (12-1493) | 173 (12-925) | 230 (13-1493) | .19 |
| Median circulating blast, % (range) | 1 (0-18) | 1 (0-16) | 1 (0-18) | .14 |
| DIPSS risk group, n (%) | ||||
| Low | 25 (13) | 1 (3) | 24 (15) | |
| Intermediate-1 | 66 (35) | 9 (28) | 57(37) | .07 |
| Intermediate-2 | 69 (37) | 14 (44) | 55 (35) | |
| High | 27 (14) | 8 (25) | 19 (12) | |
| DIPSS-plus* risk group, n (%) | ||||
| “N” evaluable = 186 | ||||
| Low | 24 (13) | 1 (3) | 23 (15) | |
| Intermediate- 1 | 26 (14) | 2 (6) | 24 (16) | .03‡ |
| Intermediate- 2 | 70 (38) | 11 (34) | 59 (38) | |
| High | 66 (35) | 18 (56) | 48 (31) | |
| Constitutional symptoms, n (%) | 68 (36) | 15 (47) | 53 (34) | .17 |
| Circulating blasts ≥ 1%, n (%) | 117 (63) | 23 (72) | 94 (61) | .23 |
| Hemoglobin < 10 g/dL, n (%) | 92 (49) | 20 (63) | 72 (46) | .10 |
| Transfusion requiring, n (%) | 61 (33) | 15 (47) | 46 (30) | .06 |
| Leukocytes > 25 × 109/L, n (%) | 36 (19) | 9 (28) | 27 (17) | .16 |
| Leukocytes < 4 × 109/L, n (%) | 27 (14) | 4 (13) | 23 (15) | .73 |
| Platelets < 100 × 109/L, n (%) | 44 (24) | 8 (25) | 36 (23) | .83 |
| JAK2V617F-positive, n (%) | 111 (59) | 23 (72) | 88 (57) | .11 |
| MPL-mutated, n (%) | 11 (6) | 0 | 11 (7) | .12 |
| “N” evaluable = 182 | ||||
| IDH1/2-mutated, n (%) | 14 (8) | 9 (28) | 5 (3) | < .0001‡ |
| “N” evaluable = 184 | ||||
| IDH1-mutated, n (%) | 6 (3) | 4 (13) | 2 (1) | .001‡ |
| “N” evaluable = 184 | ||||
| IDH2-mutated, n (%) | 8 (4) | 5 (16) | 3 (2) | .0006‡ |
| “N” evaluable = 184 | ||||
| SF3B1-mutated, n (%) | 12 (7) | 1 (4) | 11 (8) | .46 |
| “N” evaluable = 165 | ||||
| Palpable spleen > 10 cm, n (%) | 60 (32) | 14 (44) | 46 (30) | .34 |
| Splenectomized, n (%) | 28 (15) | 3 (9) | 25 (16) | .33 |
| Cytogenetic categories, n (%) | ||||
| “N” evaluable = 186 | ||||
| Normal | 113 (61) | 18 (56) | 95 (62) | |
| Favorable | 54 (29) | 12 (38) | 42 (27) | .43 |
| Unfavorable | 19 (10) | 2 (6)† | 17 (11) | |
| Transplanted, n (%) | 11 (6) | 0 | 11 (7) | .12 |
| Variable . | All patients (n = 187) . | SRSF2 mutated (n = 32) . | SRSF2 wild-type (n = 155) . | P . |
|---|---|---|---|---|
| Median age, y (range) | 61 (35-81) | 68 (39-81) | 59 (35-81) | .0004‡ |
| Age > 65 y, n (%) | 74 (40) | 20 (63) | 54 (35) | .004‡ |
| Males, % | 117 (63) | 24 (75) | 93 (60) | .11 |
| Median hemoglobin, g/dL (range) | 10 (6-15) | 10 (7-15) | 10 (6-15) | .10 |
| Median leukocytes, × 109/L (range) | 10 (1-147) | 13 (2-46) | 9 (1-147) | .08 |
| Median platelets, × 109/L (range) | 224 (12-1493) | 173 (12-925) | 230 (13-1493) | .19 |
| Median circulating blast, % (range) | 1 (0-18) | 1 (0-16) | 1 (0-18) | .14 |
| DIPSS risk group, n (%) | ||||
| Low | 25 (13) | 1 (3) | 24 (15) | |
| Intermediate-1 | 66 (35) | 9 (28) | 57(37) | .07 |
| Intermediate-2 | 69 (37) | 14 (44) | 55 (35) | |
| High | 27 (14) | 8 (25) | 19 (12) | |
| DIPSS-plus* risk group, n (%) | ||||
| “N” evaluable = 186 | ||||
| Low | 24 (13) | 1 (3) | 23 (15) | |
| Intermediate- 1 | 26 (14) | 2 (6) | 24 (16) | .03‡ |
| Intermediate- 2 | 70 (38) | 11 (34) | 59 (38) | |
| High | 66 (35) | 18 (56) | 48 (31) | |
| Constitutional symptoms, n (%) | 68 (36) | 15 (47) | 53 (34) | .17 |
| Circulating blasts ≥ 1%, n (%) | 117 (63) | 23 (72) | 94 (61) | .23 |
| Hemoglobin < 10 g/dL, n (%) | 92 (49) | 20 (63) | 72 (46) | .10 |
| Transfusion requiring, n (%) | 61 (33) | 15 (47) | 46 (30) | .06 |
| Leukocytes > 25 × 109/L, n (%) | 36 (19) | 9 (28) | 27 (17) | .16 |
| Leukocytes < 4 × 109/L, n (%) | 27 (14) | 4 (13) | 23 (15) | .73 |
| Platelets < 100 × 109/L, n (%) | 44 (24) | 8 (25) | 36 (23) | .83 |
| JAK2V617F-positive, n (%) | 111 (59) | 23 (72) | 88 (57) | .11 |
| MPL-mutated, n (%) | 11 (6) | 0 | 11 (7) | .12 |
| “N” evaluable = 182 | ||||
| IDH1/2-mutated, n (%) | 14 (8) | 9 (28) | 5 (3) | < .0001‡ |
| “N” evaluable = 184 | ||||
| IDH1-mutated, n (%) | 6 (3) | 4 (13) | 2 (1) | .001‡ |
| “N” evaluable = 184 | ||||
| IDH2-mutated, n (%) | 8 (4) | 5 (16) | 3 (2) | .0006‡ |
| “N” evaluable = 184 | ||||
| SF3B1-mutated, n (%) | 12 (7) | 1 (4) | 11 (8) | .46 |
| “N” evaluable = 165 | ||||
| Palpable spleen > 10 cm, n (%) | 60 (32) | 14 (44) | 46 (30) | .34 |
| Splenectomized, n (%) | 28 (15) | 3 (9) | 25 (16) | .33 |
| Cytogenetic categories, n (%) | ||||
| “N” evaluable = 186 | ||||
| Normal | 113 (61) | 18 (56) | 95 (62) | |
| Favorable | 54 (29) | 12 (38) | 42 (27) | .43 |
| Unfavorable | 19 (10) | 2 (6)† | 17 (11) | |
| Transplanted, n (%) | 11 (6) | 0 | 11 (7) | .12 |
DIPSS indicates Dynamic International Prognostic Scoring System.17
DIPSS-plus7 uses 8 independent predictors of inferior survival: age > 65 years, hemoglobin < 10 g/dL, leukocytes > 25 × 109/L, circulating blasts ≥ 1%, constitutional symptoms, red cell transfusion dependency, platelet count < 100 × 109/L, and unfavorable karyotype (ie, complex karyotype or sole or 2 abnormalities that include +8, −7/7q−, i(17q), inv(3), −5/5q−, 12p− or 11q23 rearrangement). The presence of 0, 1, “2 or 3,” and ≥ 4 adverse factors defines low, intermediate-1, intermediate-2, and high-risk disease, respectively.7
Unfavorable karyotype in SRSF2-mutated patients included sole abnormalities of trisomy 8 and del(7)(q22q34).
P values that were significant.