Clinical laboratory features and outcomes of the patients enrolled in the perspective outcome analysis
| . | . | JAK2 V617F nonmutated . | JAK2 V617F mutated . | P* . |
|---|---|---|---|---|
| Demographic characteristics | ||||
| Patients, no. | 174 | 88 | 86 | — |
| Male sex, n (%) | 109 (62.2) | 54 (61.4) | 55 (63.9) | NS |
| Age, y (IQR) | 54 (43-63) | 50 (39-57) | 56 (45-64) | NS |
| Follow-up, mo (IQR) | 26 (7-66) | 24 (7-84) | 28 (7-58) | NS |
| Surviving at 5 y, % (95% CI) | 81.7 (71.4-88.8) | 86.5 (73.2-91.1) | 75 (60-81.2) | .04 |
| Laboratory and clinical features at diagnosis | ||||
| Hemoglobin level, g/L (IQR) | 130 (110-140) | 117 (95-132) | 130 (110-147) | .002 |
| White cell count, × 109/L (IQR) | 8 (6-13) | 7.6 (5.4-10.7) | 9.38 (6.8-13.8) | .02 |
| Immature myeloid cells in peripheral blood, % (IQR) | 2 (0-46) | 3 (1-12) | 2 (0-7) | NS |
| Blasts in peripheral blood, % (IQR) | 0 (0-40) | 0 (1-1.5) | 4 (0-1) | NS |
| Erythroblasts in peripheral blood, % (IQR) | 1 (0-24) | 1 (0-2) | 0 (0-2) | NS |
| Platelet count, × 109/L (IQR) | 347 (197-654) | 341 (187-661) | 354 (203-653) | NS |
| Spleen index, cm2 (IQR) | 150 (100-240) | 140 (100-250) | 160 (106-230) | NS |
| Serum lactate dehydrogenase, U/L (IQR) | 730 (456-1018) | 744 (438-1003) | 730 (469-1045) | NS |
| Dupriez score at diagnosis, no. of patients (%) | ||||
| Grade 0 | 126 (72.4) | 59 (67.1) | 67 (77.9) | NS |
| Grade 1 | 37 (21.3) | 24 (27.2) | 13 (15.1) | NS |
| Grade 2 | 11 (6.31) | 5 (5.7) | 6 (7.0) | NS |
| Events during follow-up | ||||
| Patients treated with a potentially transforming drug for <6 mo at the last follow-up, no. of patients (%) | 66 (47.1) | 31 (35.2) | 35 (40.7) | NS |
| Patients submitted to splenectomy, no. of patients (%) | 29 (16.6) | 9 (10.2) | 20 (23.3) | NS |
| Major thrombotic events, no. of patients (%) | 22 (12.7) | 10 (11.4) | 12 (13.9) | NS |
| Development of large splenomegaly, no. of patients (%)† | 55 (31.6) | 24 (27.3) | 31 (36.0) | NS |
| Development of severe anemia, no. of patients (%)† | 71 (40.8) | 41 (46.6) | 30 (35.3) | NS |
| Development of thrombocytopenia, no. of patients (%)† | 57 (32.8) | 30 (34.1) | 27 (31.4) | NS |
| Development of leukopenia, no. of patients (%)† | 36 (20.9) | 23 (26.1) | 13 (15.1) | NS |
| Leukemic transformation, no. of patients (%) | 21 (12.1) | 6 (6.8) | 15 (17.4) | NS |
| Time from diagnosis to leukemic transformation, mo; median (range) | 42 (22-99) | 9 (6-17) | 47 (28-101) | NS |
| FAB classification of leukemic transformation, no. of patients (%) | ||||
| M0 | 7 (33.3) | 5 (5.7) | 2 (2.3) | NS |
| M1 | 2 (9.5) | 2 (2.3) | 0 | NS |
| M2 | 0 (0) | 0 (0) | 0 (0) | — |
| M3 | 0 (0) | 0 (0) | 0 (0) | — |
| M4 | 0 (0) | 0 (0) | 0 (0) | — |
| M5 | 3 (14.3) | 1 (1.1) | 2 (2.3) | NS |
| M6 | 3 (14.3) | 0 (0) | 3 (3.5) | NS |
| M7 | 6 (28.6) | 4 (4.5) | 2 (2.3) | NS |
| Deaths from leukemic transformation, n (%) | 12 (7) | 4 (4.5) | 8 (9.3) | NS |
| Deaths from all causes, n (%) | 21 (12.1) | 7 (7.9) | 14 (16.2) | NS |
| . | . | JAK2 V617F nonmutated . | JAK2 V617F mutated . | P* . |
|---|---|---|---|---|
| Demographic characteristics | ||||
| Patients, no. | 174 | 88 | 86 | — |
| Male sex, n (%) | 109 (62.2) | 54 (61.4) | 55 (63.9) | NS |
| Age, y (IQR) | 54 (43-63) | 50 (39-57) | 56 (45-64) | NS |
| Follow-up, mo (IQR) | 26 (7-66) | 24 (7-84) | 28 (7-58) | NS |
| Surviving at 5 y, % (95% CI) | 81.7 (71.4-88.8) | 86.5 (73.2-91.1) | 75 (60-81.2) | .04 |
| Laboratory and clinical features at diagnosis | ||||
| Hemoglobin level, g/L (IQR) | 130 (110-140) | 117 (95-132) | 130 (110-147) | .002 |
| White cell count, × 109/L (IQR) | 8 (6-13) | 7.6 (5.4-10.7) | 9.38 (6.8-13.8) | .02 |
| Immature myeloid cells in peripheral blood, % (IQR) | 2 (0-46) | 3 (1-12) | 2 (0-7) | NS |
| Blasts in peripheral blood, % (IQR) | 0 (0-40) | 0 (1-1.5) | 4 (0-1) | NS |
| Erythroblasts in peripheral blood, % (IQR) | 1 (0-24) | 1 (0-2) | 0 (0-2) | NS |
| Platelet count, × 109/L (IQR) | 347 (197-654) | 341 (187-661) | 354 (203-653) | NS |
| Spleen index, cm2 (IQR) | 150 (100-240) | 140 (100-250) | 160 (106-230) | NS |
| Serum lactate dehydrogenase, U/L (IQR) | 730 (456-1018) | 744 (438-1003) | 730 (469-1045) | NS |
| Dupriez score at diagnosis, no. of patients (%) | ||||
| Grade 0 | 126 (72.4) | 59 (67.1) | 67 (77.9) | NS |
| Grade 1 | 37 (21.3) | 24 (27.2) | 13 (15.1) | NS |
| Grade 2 | 11 (6.31) | 5 (5.7) | 6 (7.0) | NS |
| Events during follow-up | ||||
| Patients treated with a potentially transforming drug for <6 mo at the last follow-up, no. of patients (%) | 66 (47.1) | 31 (35.2) | 35 (40.7) | NS |
| Patients submitted to splenectomy, no. of patients (%) | 29 (16.6) | 9 (10.2) | 20 (23.3) | NS |
| Major thrombotic events, no. of patients (%) | 22 (12.7) | 10 (11.4) | 12 (13.9) | NS |
| Development of large splenomegaly, no. of patients (%)† | 55 (31.6) | 24 (27.3) | 31 (36.0) | NS |
| Development of severe anemia, no. of patients (%)† | 71 (40.8) | 41 (46.6) | 30 (35.3) | NS |
| Development of thrombocytopenia, no. of patients (%)† | 57 (32.8) | 30 (34.1) | 27 (31.4) | NS |
| Development of leukopenia, no. of patients (%)† | 36 (20.9) | 23 (26.1) | 13 (15.1) | NS |
| Leukemic transformation, no. of patients (%) | 21 (12.1) | 6 (6.8) | 15 (17.4) | NS |
| Time from diagnosis to leukemic transformation, mo; median (range) | 42 (22-99) | 9 (6-17) | 47 (28-101) | NS |
| FAB classification of leukemic transformation, no. of patients (%) | ||||
| M0 | 7 (33.3) | 5 (5.7) | 2 (2.3) | NS |
| M1 | 2 (9.5) | 2 (2.3) | 0 | NS |
| M2 | 0 (0) | 0 (0) | 0 (0) | — |
| M3 | 0 (0) | 0 (0) | 0 (0) | — |
| M4 | 0 (0) | 0 (0) | 0 (0) | — |
| M5 | 3 (14.3) | 1 (1.1) | 2 (2.3) | NS |
| M6 | 3 (14.3) | 0 (0) | 3 (3.5) | NS |
| M7 | 6 (28.6) | 4 (4.5) | 2 (2.3) | NS |
| Deaths from leukemic transformation, n (%) | 12 (7) | 4 (4.5) | 8 (9.3) | NS |
| Deaths from all causes, n (%) | 21 (12.1) | 7 (7.9) | 14 (16.2) | NS |
Aggregate laboratory and clinical features data are medians.
NS indicates not significant; IQR, interquartile range; and —, not applicable.
Statistical significance of the difference between JAK2 V617F mutated and nonmutated patients.
Development of large splenomegaly was intended as spleen tip extending greater than 10 cm from left costal margin, development of severe anemia was intended as hemoglobin level less than 100 g/L, development of thrombocytopenia was intended as platelet count lower than 150 x109/L, development of leukopenia was intended as white blood cell count lower than 4 × 109 /L.
The genotype at the time of leukemic transformation was measured on the DNA purified from granulocytes. In 12 of the patients, CD34+ cells from peripheral blood were separated, and V617F mutation was detected on the separated cells. In all the cases, CD34+ cells had the same genotype of granulocytes.